61 research outputs found
Orthopedic evolution of patient with Duchenne muscular dystrophy from reference center of Lille university hospital
Effets de la fampridine sur les paramètres de marche, la fonction des membres supérieurs, la fatigue et la qualité de vie chez les patients atteints de sclérose en plaques
Promoting the use of Motor Function Measure (MFM) as outcome measure in patients with Duchenne Muscular Dystrophy (DMD) treated by corticosteroids
ObjectivesAssessing muscle function is a key step in measuring changes and evaluating the outcomes of therapeutic interventions in Duchenne Muscular Dystrophy (DMD). Regarding the large use of corticosteroids (CS) in this population to delay the loss of function, our goal was to monitor the evolution of motor function in patients with DMD treated by corticosteroids (CS) and to study the responsiveness of Motor Function Measure (MFM) in this population in order to provide an estimation of the number of subject needed for a clinical trial.MethodA total of 76 patients with DMD, aged 5.9 to 11.8 years, with at least 6 months of follow-up and 2 MFM were enrolled, 30 in the CS treated group (8±1.62 y) and 46 in the untreated group (7.91±1.50 y).ResultsThe relationship between MFM scores and age was studied in CS treated patients and untreated patients. The evolution of these scores was compared between groups, on a 6-, 12- and 24-month period by calculating slopes of change and standardized response mean. At 6, 12 and 24 months, significant differences in the mean score change were found, for all MFM scores, between CS treated patients and untreated patients. For D1 subscore specifically, at 6 months, the increase is significant in the treated group (11.3±14%/y; SRM 0.8) while a decrease is observed in the untreated group (–17.8±17.7%/y; SRM 1). At 12 and 24 months, D1 subscore stabilized for treated patients but declined significantly for untreated boys (–15.5±15.1%/y; SRM 1 at 12 mo and–18.8±7.1%/y; SRM 2.6 at 24 mo). 21 patients lost the ability to walk during the study: 6 in the CS treated group (25% at 24 months, mean age: 10.74±1.28 y) and 15 in the untreated group (64.71% at 24 months, mean age: 9.20±1.78 y).Discussion and conclusionPatients with DMD treated by CS present a different course of the disease described in this paper using the MFM. Based on these results, an estimation of the number of patients needed for clinical trial could be done
Thiol-functionalized magnetite/graphene oxide hybrid as a reusable adsorbent for Hg2+ removal
Évolution orthopédique des patients atteints de dystrophinopathie de Duchenne du centre de référence neuromusculaire du CHRU de Lille
Orthopedic evolution of patient with Duchenne muscular dystrophy from reference center of Lille university hospital
Evaluation of muscle oxygenation by near-infrared spectroscopy in patients with Becker muscular dystrophy
Abstract Several authors have reported alterations in vasodilation during effort in patients with dystrophinopathies, in which a lack of neuronal NO synthase is thought to lead to functional muscle ischemia. In order to determine changes in muscle oxygenation during effort in patients with Becker muscular dystrophy (BMD) and assess the parameters' links with disease severity and functional status, 10 BMD patients and 10 age-matched controls performed isokinetic, constant-load knee extension exercises at (i) 20% of their extensors' peak torque (i.e. the same relative load) and (ii) the same absolute load (20 Nm). Muscle oxygenation was evaluated noninvasively using near-infrared spectroscopy (NIRS), with the time course of deoxygenation as the main criterion. As expected, BMD patients displayed a lower peak torque than controls (Ă€62%). During both types of exercise, initial muscle deoxygenation was faster (by 27-41%) in BMD patients than in controls. Greater disease severity (according to the Motor Function Measure) and functional impairment (walking endurance) were associated with a faster second deoxygenation phase (s). The validity and relevance of muscle deoxygenation parameters and the alteration of vasodilatation by nNOS deficiency in dystrophinopathies should be assessed by further studies
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