source: Neuromuscular Disorders
E. Zapata Aldana, N. Eltayeb, M. Miller, C. Campbell
Duchene muscular dystrophy (DMD) is the most common form of childhood muscular dystrophy. Clinically DMD is characterized by early onset of progressive muscle weakness, and losing ambulation at a mean age of 9.5 years in patients without corticosteroid regime. The purpose of this study was to examine the hypothesis that short stature is associated with the preservation of ambulation in DMD patients. Data was obtained from the Canadian Neuromuscular Disease Registry, a Canada-wide registry. The database included: patients with DMD, ages between 7–18 years, height, weight, wheelchair use, and corticosteroid treatment. Our primary outcome was ambulation status defined by never, intermittent, and permanent use of wheelchair. The data were analyzed using SPSS v.24. Data were obtained for 180 males with DMD. Mean (SD) age at evaluation was 12.62 (+/-3.25) years old; 47.2% had height-for-age < -2SD; 28.9% had BMI-for-age > +2SD; 90.6% were treated with a corticosteroid at the time of the visit; and 23.3%, 34.4%, and 42.2% of patients never, intermittently, and permanently used a wheelchair, respectively. A positive relation was found between wheelchair use and the following variables: age, height, weight, BMI, and corticosteroid use. However, age was the only significant predictor of wheelchair use in the multivariable model. Although in univariate analysis shorter stature correlated with preserved ambulation status, in multivariable modeling, age was the only significant predictor of ambulation. We are currently in the process of examining longitudinal data to see how temporal changes in stature impact ambulation within DMD patients.
Western University, Canada
10.1016/j.nmd.2017.06.038 read more