Primary Disease Fascioscapulohumeral Dystrophy (FSHD)
Conditions
Keywords
FSHD, Usual care performed at Montpellier hospital, Muscle function, Antioxidants, Oxidative stress, Quality of life, Physical activities, development of food-based dietary guidelines, Co-morbidity
Brief summary
On the basis of published data and our results indicating that oxidative stress may contribute to the peripheral skeletal muscle dysfunction in patients with FSHD, investigators conducted a pilot randomized double-blind placebo controlled trial to test whether oral administration of vitamins and mineral could improve the physical performance of patients with FSHD.The result of this clinical trial showed that antioxidants supplementation may improve skeletal muscle function of patients with FSHD and suggest that an antioxidant strategy adapted may be a relevant therapeutic approach for these patients. Since then, patients with FSHD who attend consultation at Montpellier hospital are systematically supplemented with antioxidants according their own blood tests.
Detailed description
This study will assess course of patients with FSHD in accordance with usual care performed within the Department of clinical physiology at the University hospital of Montpellier. This prospective longitudinal study include an initial assessment. Then the follow up of patients will be monitored at 12 months and annually. The eventual objective will be to develop a FSHD disease cohort in care at the Montpellier hospital. A statistical analysis will be performed at 3-years follow-up
Interventions
OTHERFollowed with an initial assessment, 12 months and followed annually corresponding to the usual follow-up of the patient.
Sponsors
University Hospital, Montpellier
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
7 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Molecular and clinical diagnosis for FSHD * patients, men and women aged \> 7 years,
Exclusion criteria
* pregnant women * Inability to understand the nature and goals of the study and / or communication difficulties with the investigator * Major protected by law (guardianship, curatorship or under judicial protection)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Effect of usual care performed at Montpellier hospital on the measurement of the maximal voluntary contraction (MVC) of quadriceps expressed in kg | 1 day (the day of inclusion) |
Secondary
| Measure | Time frame |
|---|---|
| Effect of usual care performed at Montpellier hospital on quality of life (SF36 questionnaire) | 1 day (the day of inclusion) |
| Effect of usual care performed at Montpelier hospital on physical activities (Vorrips questionnaire) | 1 day (the day of inclusion) |
| Effect of usual care performed at Montpellier hospital on physical activities (Vorrips questionnaire) | 12 months after inclusion |
| Effect of usual care performed at Montpellier hospital on oxidative stress markers measured by laboratory tests | 1 day (the day of inclusion) |
Countries
France
Outcome results
None listed