Duchenne Dystrophy Muscular
Conditions
Keywords
DMD, morphological predictive factors, Mechanical
Brief summary
The loss of ability to walk in many children with DMD (Duchenne muscular Dystrophy) is a pejorative event. Biomechanical and morphological unknowledge about the loss of the walk ability in children with DMD is an obstacle in reeducative, pharmacological or surgical therapeutic targets.
Detailed description
The loss of ability to walk in many children with DMD (Duchenne muscular Dystrophy) is a pejorative event. Biomechanical and morphological unknowledge about the loss of the walk ability in children with DMD is an obstacle in reeducative, pharmacological or surgical therapeutic targets. We suppose that there are muscular characteristics and predictive parameters of the loss of walk ability. The identification of these potential therapeutic targets would improve the surveillance and the clinical care but would also guide future clinical and fundamental trials too.
Interventions
OTHERNo drug and no placebo were used in this study
measures of muscular strength of legs, motor function, walk testing and analysis (3D video recording), RMI
Sponsors
University Hospital, Brest
Study design
Allocation
NON_RANDOMIZED
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
5 Years to 17 Years
Healthy volunteers
Yes
Inclusion criteria
* Young man ou woman (5 to 17 years old) with Duchenne Muscular Dystrophy (confirmed by immunohistochimy on the muscular biopsy and/or mutation in the dystrophin confirmed by molecular biology) * Time more than 7 secondes to test of 10 m and/or distance less than 330 m to walk test of 6 minutes. These values are recent markers to include children with a strong risk of loss of walking ability in 2 years. * Parental inform sign consent and / or child inform consent
Exclusion criteria
* Recent orthopaedic surgery of lower limbs (6 months) * Other chronic disease associated, which have an impact on the walking * Cognitive Deficiency or behavior disorders limiting the understanding of the study * Children who can benefit ATU (translarna ® or other) during the study * All MRI contradications : pacemaker or neurosensory stimulator or implantable defibrillator, neurosurgical valves, cochlear implant or ferromagnetic implants near nervous structures, brace, metallic prostheses, not cooperative or agitated patients, patient claustrophobic, pregnant woman.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Determine biomechanical and morphological predictive factors of the loss of the walk ability of the children with DMD | 24 years | Determine the biomechanical factors (muscular atrophy, muscular strength, muscular shrinkage) and morphological (greasy infiltration, contractile portion, muscular geometry) predictive of the loss of the walk ability of the children with DMD |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Identify the muscles wasting and their implication in the loss of strength and the walking ability | 24 years | Identify the muscles wasting and their implication in the loss of strength and walking. This will be made possible thanks to the predictive analysis and the evolutionary analysis of the MRI and strength before and after loss of walking ability |
| Biomechanical evolutionary data collected during the last 2 years of walking | 24 years | Biomechanical evolutionary data collected during the last 2 years of walking by the repetition of the analysis of the walking until the loss of the walking ability. |
| Establish the relationship between the parameters of walking and the scrawny body morphological anomalies | 24 years | Establish the relationship between the parameters of walking and the scrawny body morphological anomalies (muscular Atrophy, greasy infiltration, contractile muscular portion, three-dimensional morphological parameters) by doing correlations analysis |
Countries
France
Contacts
Primary ContactSylvain BROCHARD, Dr
Outcome results
None listed