MUSCLE FIBRE SIZE AND SHAPE IN DUCHENNE MUSCULAR DYSTROPHY

Abstract
A method for measuring fiber area and shape which obviates variabilities inherent in diameter measuring techniques was applied to dystrophic and normal human muscle. The mean fiber area in patients with Duchenne muscular dystrophy (DMD) increases with age at a greater rate than that of the controls until .apprx. 5 yr of age, when it decreases rapidly. This is due to an increasing population of small regenerating, regenerated or split fibers. The shape of fibers was expressed as a form factor and it was seen that the fibers measured from DMD biopsies were less regular in shape than the controls due to constraints placed on the normal growth of regenerating fibers by the proliferation of connective tissue.