[Investigation of mast cells association with muscle fibrosis in patients with Duchenne muscular dystrophy]

ABSTRACT

Duchenne muscular dystrophy [DMD] is the most common and severe type of muscular dystrophy which occurs due to the absence of the cytoskeletal protein of dystrophin. The gene that is responsible for DMD was localized in the XP21 of human genum. Severe weakness of the skeletal muscles seen in the DMD patients seemed to be due to the fibrosis, but the reasons for it were not revealed. It was shown that in lung fibrosis and scleroderma,] mast cells intervene with fibrosis. Thus, we decided to evaluate the relationship between fibrosis and number of the mast cells in DMD patients. This research was done on 51 DMD patients and due to clinical findings, we classified them into two groups mild and severe. Having done different staining on sections of paraffin blocks of the patients muscle biopsies, we evaluated the degree of fibrosis and the number of mast cells. Following by T-Test statistical method, the relationship between degree of fibrosis and number of mast cells was found. Therefore, it could be concluded that mast cells are responsible for mild and moderate fibrosis