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40
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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.
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URL Address
<a href="http://doi.org/10.1017/s0012162206001083" target="_blank" rel="noreferrer">http://doi.org/10.1017/s0012162206001083</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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Management of scoliosis in Duchenne muscular dystrophy: a large 10-year retrospective study
Publisher
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Developmental Medicine & Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2006
Subject
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PedPal Lit; Adolescent Comparative Study Decision Making Disability Evaluation Female Humans Male Muscular Dystrophy; Duchenne/complications Postoperative Period Research Support; Non-U.S. Gov't Retrospective Studies Scoliosis/etiology/surgery/therapy Time Factors Treatment Outcome
Creator
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Kinali M; Messina S; Mercuri E; Lehovsky J; Edge G; Manzur AY; Muntoni F
Description
An account of the resource
Scoliosis affects 75 to 90% of patients with non-ambulant Duchenne muscular dystrophy (DMD). Spinal surgery is the treatment of choice but the indication varies among centres. Some offer surgery to all non-ambulant patients, irrespective of scoliosis severity. Early surgery has the advantage of targeting DMD when cardiorespiratory function is preserved, but not all patients develop scoliosis. We report our 10-year experience of scoliosis management in 123 patients with DMD who were at least 17 years old at the time of the study. Scoliosis was absent in 10%, and mild, non-progressive (at least 30 degrees ) in 13% of patients. Another 13% had moderate scoliosis (31-50 degrees ) and were managed conservatively. Surgery was considered in 57% (70/123) of patients with scoliosis greater than 50 degrees and eventually performed in 35%. The remaining patients either refused surgery (9%) or were unfit because of cardiorespiratory compromise (13%). In a further 7%, scoliosis (greater than 50 degrees ), first noted after 14 years of age, was progressing slowly and surgery was not performed. At 17 years there was no difference in survival, respiratory impairment, or sitting comfort among patients managed conservatively or with surgery. One-third (44/123) of our patients were managed satisfactorily without receiving spinal surgery. We provide insight into the natural history of scoliosis in DMD that should help families and clinicians with decision-making when surgery is considered.
2006
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1017/s0012162206001083" target="_blank" rel="noreferrer">10.1017/s0012162206001083</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).
Type
The nature or genre of the resource
Journal Article
2006
Adolescent Comparative Study Decision Making Disability Evaluation Female Humans Male Muscular Dystrophy
Backlog
Developmental Medicine & Child Neurology
Duchenne/complications Postoperative Period Research Support
Edge G
Journal Article
Kinali M
Lehovsky J
Manzur AY
Mercuri E
Messina S
Muntoni F
Non-U.S. Gov't Retrospective Studies Scoliosis/etiology/surgery/therapy Time Factors Treatment Outcome
PedPal Lit
-
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.
Citation List Month
Backlog
URL Address
<a href="http://doi.org/10.1136/adc.2007.118141" target="_blank" rel="noreferrer">http://doi.org/10.1136/adc.2007.118141</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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Update on the management of Duchenne muscular dystrophy
Publisher
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Archives Of Disease In Childhood
Date
A point or period of time associated with an event in the lifecycle of the resource
2008
Subject
The topic of the resource
Humans; Male; patient care team; Cardiomyopathy; Muscular Dystrophy; Physical Therapy Modalities; Respiration Disorders/etiology/therapy; Dilated/etiology/therapy; Duchenne/complications/diagnosis/genetics/therapy; Dystrophin/genetics; Gene Therapy/methods; Glucocorticoids/therapeutic use; Scoliosis/etiology/therapy
Creator
An entity primarily responsible for making the resource
Manzur AY; Kinali M; Muntoni F
Description
An account of the resource
Duchenne muscular dystrophy (DMD) is familiar to paediatricians as the most common childhood muscular dystrophy and leads to severe disability and early death in the late teenage years if untreated. Improvements in general care, glucocorticoid corticosteroid treatment, non-invasive ventilatory support, and cardiomyopathy and scoliosis management have significantly changed the course of DMD in treated individuals, so that survival into adulthood is now a realistic possibility for most patients. This has important implications for the medical and social sectors involved in the transition to adult medical services and the provision of suitable employment and social care. Multidisciplinary team working for optimal management of DMD-specific multisystem complications is essential, and collaboration in disease specific national clinical networks is recommended. Several curative therapeutic strategies including cell and gene therapy are being pursued but are still at an experimental stage.
2008
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1136/adc.2007.118141" target="_blank" rel="noreferrer">10.1136/adc.2007.118141</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).
Type
The nature or genre of the resource
Journal Article
2008
Archives of Disease in Childhood
Backlog
Cardiomyopathy
Dilated/etiology/therapy
Duchenne/complications/diagnosis/genetics/therapy
Dystrophin/genetics
Gene Therapy/methods
Glucocorticoids/therapeutic use
Humans
Journal Article
Kinali M
Male
Manzur AY
Muntoni F
Muscular Dystrophy
Patient Care Team
Physical Therapy Modalities
Respiration Disorders/etiology/therapy
Scoliosis/etiology/therapy