الفهرس 
Muscular dystrophyOnly 14 pages are availabe for public view
 |  | from | 210 |  |  |
| | | from | 210 | | |
Abstract
Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy affecting 1 in 3500 boys born worldwide. It is an x-linked recessive disorder caused by mutations in dystrophin gene. It is characterized by proximal muscle weakness and calf hypertrophy in affected boys. Patients usually become wheelchair-bound by the age of 12 years, and die of cardio-respiratory complications in their late teens to early twenties.
Duchenne muscular dystrophy (DMD) is characterized by increased muscle damage and an abnormal blood flow after muscle contraction leading to a state of functional ischemia.
Normal muscle function relies on maintenance and regeneration of myofibers, which is a highly regulated process beginning with activation of quiescent muscle precursor cells, which then form proliferating progenitors that fuse to form differentiated myofibres.
Stromal cell-derived factor (SDF)-1 plays an important role in neovascularization. (SDF)-1 is an EPC chemokine known to be responsible for both progenitor cell mobilization from the bone marrow to peripheral blood and homing to the sites of vascular and tissue injury.
SDF1-CXCR4 axis affects migration, proliferation and differentiation of myogenic precursors and myoblasts. This, together with the previous observations of a massive impairment of fetal limb-muscle development in CXCR4-deficient animals, point to a prime role of SDF1- CXCR4 in the secondary expansion of muscle masses.
Several studies suggest that circulating stem cell populations as hematopoietic stem and progenitor cells and endothelial progenitor cells which are present in peripheral blood and can be identified by the presence of the CD34, CD133 and KDR antigen on the cell surface participate in the regeneration of the host tissues.
The aim of the present work is to measure plasma level of Stromal Cell-Derived factor 1(Sdf1) and to measure CD34, CD133 and KDR mononuclear cell surface marker in an attempt to investigate markers of regeneration in blood of DMD patients as a markers of muscular regeneration in patients with DMD.
The study included 25 DMD patients and 20 age and soscioeconomic matching healthy controls. All patients included in this study were subjected to full medical history, throughout clinical examination, Echocardiography. Laboratory investigations including determination of plasma creatine kinase, flow Cytometry analysis of CD34+, CD133+ , KDR membrane markers on circulating mononuclear cells and plasma Stromal Cell-Derived factor 1(Sdf1) were conducted for both patients and controls.
Plasma level of creatinine kinase was significantly higher among DMD patients compared to controls, indicating that patients in the present study represent typical DMD cases.
The plasma level of Stromal Cell-Derived factor 1(Sdf1) was significantly higher among DMD patients compared to controls.
The mononuclear cell expressing CD34 surface marker was significantly higher among DMD patients compared to controls.
The mononuclear cell expressing CD133 surface marker was significantly higher among DMD patients compared to controls.
The mononuclear cell expressing KDR surface marker was significantly higher among DMD patients compared to controls.
There was a negative correlation between plasma CPK and muscle function in DMD patients.
There was a positive correlation between muscle function and SDF1, CD34, CD133, KDR in DMD patients.
There was a positive correlation between age and plasma CPK in DMD patients.
There was a negative correlation between age and SDF1, CD34, CD133, KDR in DMD patients.
There was a positive correlation between plasma CPK and SDF1, CD34 in DMD patients.
There was a positive non significant correlation between plasma CPK and mononuclear cell expressing CD133 surface marker in DMD patients.
There was a negative non significant correlation between plasma CPK and mononuclear cell expressing KDR surface marker in DMD patients.