Lung function in children with Duchenne's muscular dystrophy

Abstract

Duchenne's muscular dystrophy (DMD), characterized by gradually developing muscular weakness, leads to respiratory symptoms and reduced lung function. We aimed to assess lung function in 25 patients with DMD in relationship to age and muscular function. The 25 boys, mean age 13 years, comprized patients in southern Norway with DMD, taking part in an epidemiological follow-up study. None had chronic respiratory disease. Lung function was measured by maximum expiratory flow-volume loops and whole body plethysmography, and repeated after 1 year (n= 14). Lung function was reduced compared to predicted values for healthy children. Forced expiratory volume in 1 sec (FEV1)% predicted and forced vital capacity (FVC)% predicted correlated (significantly) inversely to age. FEV1 and FVC decreased annually 5.61 and 4.2% of predicted, respectively. Absolute values of FVC (litres) and FEV1 (1 sec(-1)) increased until mean age 14 years, decreasing thereafter. Values in % predicted decreased steadily throughout the age range (6-19 years). Lung function correlated closely to upper limb muscle function.