Abstract
DMD is characterized by progressive weakness of striated muscles accompanied by spinal deformity and deterioration of pulmonary function, the main cause of death.Scoliosis progresses with age becoming one of the major complication but there are still conflicting reports on the effect of SF on improving respiratory function in DMD.Since these studies considered only spirometry,we aimed to investigate if also spontaneous breathing is affected as well as vital capacity.We computed the Spearman correlation coefficient (RS) to quantify how strongly scoliosis and SF are associated with minute ventilation (VE) at rest in supine position and with forced vital capacity percentage predicted (FVC%). The analysis was made on a total of 597 recordings obtained in the past 6 years from 95 DMD patients grouped according to age (6-9,10-13,14-17,18-21,22-24 years).Scoliosis was classified null (N, Cobb angle=0°), mild (MI, <20°), moderate (M, 20-40°) and severe (S,>40°). In the groups 10-13, 14-17 and 18-21, RS was about -0.2 between scoliosis and VE, while RS was <-0.49 between scoliosis and FVC%, with SF being the nadir (figure).VE is not related with the spine curvature unlikely to FVC% that decreased with the severity of scoliosis and worsened after SF.Scoliosis affected respiratory function only during maximal maneuver,while SF is not an ameliorative treatment of the restrictive DMD lung.
- Copyright ©ERS 2015