چاپ صفحه |  صفحه نخست سامانه |  نویسندگان |  اطلاعات تفضیلی |  دانلود مقاله | دانشگاه علوم پزشکی تبریز |
| نویسنده | نفر چندم مقاله |
|---|---|
| امیررضا جهانشاهی | اول |
| عنوان | متن |
|---|---|
| کلمات کلیدی | Osteogenesis imperfecta, cleidocranial dysplasia, sclerosing bone dysplasia, dwarfism, mucopolysaccharidosis |
| خلاصه مقاله | Skeletal dysplasia, main types and differentiation Osteogenesis Imperefecta: Disorders of collagen synthesis that result in abnormal bone formation with radiolucent bones that are easily fractured. Presentation is include hearing loss (caused by otic bone fractures), gray teeth (dentinogenesis imperfecta), and blue sclera, osteoporosis, and multiple wormian bones formation. Sillence classification is done for these patients, based on Relative Bone Fragility, Bone Deformity, Stature, Blue Sclera, Dentinogenesis Imperfecta, hearing Loss and Inheritance pattern. Cleidocranial Dysplasia: Usually evident at birth and may be detected-prenatally, presented by small face, wide head with hypertelorism, and generalized joint laxity. Patinets have delayed closure of the cranial sutures and fontanelles, including a persistent metopic suture, Wormian bones and also partial or complete absence of the clavicle with occasional apparent pseudarthrosis. Sclerosing bone Dysplasias: A varied group of conditions that have common various patterns of increased bone density. Some are incidental finding and asymptomatic but others have fragile bones that are easily fractured. Caffey disease: disease presentation is with exuberant periosteal new bone formation in infants, especially around the mandible. Osteopetrosis: This is due to diminished osteoclast function, patient's presentation are with diffusely very dense but brittle bone s and with broad, dense metaphyseal bands, they may have bone-within-a-bone appearance. Pykondysostosis: Patients present with short-limbed dwarfism with diffuse osteosclerosis and frequent transverse fracture of long bone. Melorheostosis: In these patients, danse bone is deposited along the cortex of otherwise normal bones and has Dripping candle wax pattern (irregular, elongate and wavy). Dwarfism: Group of disorders that present with disproportionate limb or spine shortening that results in short stature. Screening radiography in a child with a dwarfism should include: lateral skull, anteroposterior (AP) and lateral thoracolumbar spine, frontal chest (including the shoulders), AP pelvis and hips, AP view of a single upper and lower extremity, and posteroanterior hand view. Achondroplasia: One of the most common dwarfisms. Intelligence and life span are normal in these patients. Vertebral bodies are bullet shaped in infancy and become mildly flattened in adulthood. Major cause of morbidity in these patients is neurologic impingement caused by the spinal and cranial stenosis. Mucoplolysaccharidosis: This disorder is due to accumulation of mucopolysaccharides in the bone marrow, brain, liver, and other organs. It calcified by differences in clinical manifestations, biochemistry, and inheritance. A group of skeletal manifestations, that are seen at MPS is called Dysostosis multiplex. Hurler syndrome (MPS IH), is the most common type of MPS, it causes early and severe appearance of dysostosis multiplex (beginning by about 1 year of age), with death resulting in first decade of life, often from cardiac disease. Morquio syndrome: this is only (relatively) common type of MPS that does not cause mental retardation. |
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| 1 001.jpg | 1401/02/28 | 1432690 | دانلود |
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| 1 003.jpg | 1401/02/31 | 828291 | دانلود |
