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Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy

PURPOSE: In the 1980s the first results of an early multilevel contracture release (MLCR) in patients suffering from progressive Duchenne muscular dystrophy (DMD) showed a positive effect on ambulation. Despite the demonstrated positive effects of prolongation of walking this treatment is not part o...

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Autores principales: Weiß, Claudia, Stoltenburg, Corinna, Bayram, Dilan, Funk, Julia, Lebek, Susanne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The British Editorial Society of Bone & Joint Surgery 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7453179/
https://www.ncbi.nlm.nih.gov/pubmed/32874370
http://dx.doi.org/10.1302/1863-2548.14.200033
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author Weiß, Claudia
Stoltenburg, Corinna
Bayram, Dilan
Funk, Julia
Lebek, Susanne
author_facet Weiß, Claudia
Stoltenburg, Corinna
Bayram, Dilan
Funk, Julia
Lebek, Susanne
author_sort Weiß, Claudia
collection PubMed
description PURPOSE: In the 1980s the first results of an early multilevel contracture release (MLCR) in patients suffering from progressive Duchenne muscular dystrophy (DMD) showed a positive effect on ambulation. Despite the demonstrated positive effects of prolongation of walking this treatment is not part of current guidelines. The aim of our study was to evaluate the effect of MLCR as well as its combination with glucocorticoid (GC) treatment on ambulation. METHODS: Data of all boys (n = 86) with DMD treated in our outpatient department were analyzed regarding the treatment and loss of independent ambulation. In all, 23 were treated with GC only, ten were operated on, 21 received GC and underwent MLCR and 32 received neither of the two treatments. RESULTS: The analysis of the loss of independent ambulation in our cohort showed a comparable extension of the ambulatory period between the GC-treated and MLCR-treated boys (p = 0.008 and p = 0.005, respectively). Furthermore, an additive effect of both therapies was found; patients with DMD who had both treatments were able to walk two years longer than those with only one of the two treatment options (p<0.001). CONCLUSION: Standard GC treatment and early MLCR in lower limbs have an independent positive effect on prolongation of ambulation in patients with DMD. In our cohort, the combination of both therapies is significantly more effective than each therapy alone. We suggest both should be offered to all DMD patients eligible. Level of evidence: III
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spelling pubmed-74531792020-08-31 Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy Weiß, Claudia Stoltenburg, Corinna Bayram, Dilan Funk, Julia Lebek, Susanne J Child Orthop Original Clinical Article PURPOSE: In the 1980s the first results of an early multilevel contracture release (MLCR) in patients suffering from progressive Duchenne muscular dystrophy (DMD) showed a positive effect on ambulation. Despite the demonstrated positive effects of prolongation of walking this treatment is not part of current guidelines. The aim of our study was to evaluate the effect of MLCR as well as its combination with glucocorticoid (GC) treatment on ambulation. METHODS: Data of all boys (n = 86) with DMD treated in our outpatient department were analyzed regarding the treatment and loss of independent ambulation. In all, 23 were treated with GC only, ten were operated on, 21 received GC and underwent MLCR and 32 received neither of the two treatments. RESULTS: The analysis of the loss of independent ambulation in our cohort showed a comparable extension of the ambulatory period between the GC-treated and MLCR-treated boys (p = 0.008 and p = 0.005, respectively). Furthermore, an additive effect of both therapies was found; patients with DMD who had both treatments were able to walk two years longer than those with only one of the two treatment options (p<0.001). CONCLUSION: Standard GC treatment and early MLCR in lower limbs have an independent positive effect on prolongation of ambulation in patients with DMD. In our cohort, the combination of both therapies is significantly more effective than each therapy alone. We suggest both should be offered to all DMD patients eligible. Level of evidence: III The British Editorial Society of Bone & Joint Surgery 2020-08-01 /pmc/articles/PMC7453179/ /pubmed/32874370 http://dx.doi.org/10.1302/1863-2548.14.200033 Text en Copyright © 2020, The author(s) http://creativecommons.org/licenses/by-nc/4.0/ Open Access This article is distributed under the terms of the Creative Commons Attribution-Non Commercial 4.0 International (CC BY-NC 4.0) licence (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed.
spellingShingle Original Clinical Article
Weiß, Claudia
Stoltenburg, Corinna
Bayram, Dilan
Funk, Julia
Lebek, Susanne
Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title_full Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title_fullStr Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title_full_unstemmed Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title_short Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy
title_sort positive effect of the combination of multilevel contracture release and glucocorticoid treatment in duchenne muscular dystrophy
topic Original Clinical Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7453179/
https://www.ncbi.nlm.nih.gov/pubmed/32874370
http://dx.doi.org/10.1302/1863-2548.14.200033
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