[Evaluation of the respiratory function in patients with Duchenne's muscular dystrophy]

Abstract

The purpose of this investigation was to compare the respiratory function of patients with different clinical stages of Duchenne muscular dystrophy (DMD). Twenty-three DMD patients who were followed at Paediatric Department of Florence, were studied. We found a good correlation between the subject's functional capacity and normal predicted values for forced vital capacity (FVC) as well for forced volume at first second (FEV1). Subjects whose FVCs ranging from 10% to 20% were considered mildly involved; subjects with FVCs ranging from 20 to 30% were considered moderately involved, severe involved subjects with FVCs ranging from 30% to 40%. When the FVCs were less than 40% of the predicted values, the subjects were considered to have very severe respiratory impairment. In our study 83% of patients wheelchair bound was characterised by a very severe deterioration of pulmonary function.