Children with myositis ossificans.docVIP

 PAGE \* MERGEFORMAT 22 Children with myositis ossificans Myositis ossificans progressiva (myositis ossificans progressiva, MOP) also known as fibrous dysplasia with progressive resistance (fibrodysplasia ossificans progressiva, FOP), is currently reported about 600 cases of foreign and domestic reporting less. The disease is autosomal dominant genetic disease [1 ~ 5,9], clinical manifestations of specific skeletal deformities (mainly short big toe, and valgus) into fascia, ligament, skeletal muscle to bone, resulting in joint activities restricted as well as loss of function. The disease mostly childhood-onset. An etiology and pathogenesis of At present studies suggest that bone morphogenetic protein 4 (bone morphogenetic proteins 4, BMP-4) and its mRNA, the Myositis ossificans progressiva. BMP-4 is a protein, its gene located on chromosome 14q22-q23 [6], in physiological circumstances, BMP-4 can induce embryonic undifferentiated mesenchymal cells to contain bone, cartilage and connective tissue of bone and joint system. In the FOP patient’s lymphocytes and lesion area into cells, BMP-4 and its mRNA levels higher than the normal control group, therefore, BMP-4 overexpression in Myositis ossificans progressiva of heterotopic ossification related to [7 , 8]. Olmsted [9] for an FOP family members of lymphocytes into the steady-state BMP-4 messenger RNA quantitative analysis (semi-quantitative RT-PCR method), test data show that the mother is not sick although BMP-4 mRNA could be detected But out of low value while the ailing father and three children, BMP-4 mRNA level of 50 ~ 220 times the mother, the father of the BMP-4 mRNA levels were significantly higher than that of each child; Olmsted made a further research, from the FOP patients and normal cells isolated nuclei, the former nuclear transcription efficiency of BMP-4 mRNA is higher than the latter; He also measured the FOP patients and normal half-life of BMP-4 mRNA and found that the former half-l