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Fibrodysplasia ossificans progressiva (/ ˌ f aɪ b r oʊ d ɪ ˈ s p l eɪ ʒ (i) ə ɒ ˈ s ɪ f ɪ k æ n z p r ə ˈ ɡ r ɛ s ɪ v ə /; [1] abbr. FOP), also called Münchmeyer disease or formerly myositis ossificans progressiva, is an extremely rare connective tissue disease in which fibrous connective tissue such as muscle, tendons, and ligaments turn into bone tissue (ossification).
This mutation causes bone deformities, fractures, and delayed tooth eruption. [73] Type XIII – OI caused by a mutation in the bone morphogenetic protein 1 (BMP1) gene on chromosome 8p21.3. [74] This mutation causes recurrent fractures, high bone mass, and hypermobile joints. [75] Type XIV – OI caused by mutations in the TMEM38B gene on ...
Fibrous dysplasia is a very rare [2] nonhereditary genetic disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. As a result, most complications result from fracture , deformity, functional impairment, pain, and the impingement of nerves. [ 3 ]
This is usually due to impaired bony healing, for example due to vascular causes (e.g. impaired blood supply to the bone fragments) or metabolic causes (e.g. diabetes or smoking). Failure of initial union, as when bone fragments are separated by soft tissue, may also lead to an atrophic non-union. Atrophic non-unions can be treated by ...
Fibrous dysplasia causes bone thinning [13] and growths or lesions in one or more bones of the human body. These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions ...
Bone tissue is removed by osteoclasts, and then new bone tissue is formed by osteoblasts. Both processes utilize cytokine (TGF-β, IGF) signalling.In osteology, bone remodeling or bone metabolism is a lifelong process where mature bone tissue is removed from the skeleton (a process called bone resorption) and new bone tissue is formed (a process called ossification or new bone formation).
Osteolysis is an active resorption of bone matrix by osteoclasts and can be interpreted as the reverse of ossification.Although osteoclasts are active during the natural formation of healthy bone the term "osteolysis" specifically refers to a pathological process.
The initiation of endochondral ossification starts by proliferation and condensation of mesenchymal cells in the area where the bone will eventually be formed. Subsequently, these mesenchymal progenitor cells differentiate into chondroblasts, which actively synthesize cartilage matrix components.