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Marble bone disease

机译:大理石骨病

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摘要

Albers-Schbnberg first reported osteopetrosis in 1904 in an adult patient with generalized bone sclerosis and multiple fractures.1 Osteopetrosis is a heterogeneous group of inherited bone remodeling disorders in which there is a defect in bone resorption by osteoclasts. The disease is associated with an increase in skeletal mass due to abnormally dense bone. The decrease in osteo-clastic activity also affects the shape and structure of the bone. Abnormal remodeling of primary woven bone to lamellar bone results in brittle bone that is prone to fracture.
机译:Albers-Schbnberg于1904年首次报道了一名患有全身性骨硬化和多发性骨折的成年患者的骨质疏松症。1骨质疏松症是一组异质性遗传性骨重塑疾病,其中破骨细胞的骨吸收存在缺陷。该疾病与由于异常致密的骨骼引起的骨骼质量增加有关。破骨细胞活性的降低也影响骨骼的形状和结构。初级编织骨向板状骨的异常重塑会导致脆性骨断裂。

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