The morbid anatomy of the skeleton in osteogenesis imperfecta.
The morbid anatomy of the skeleton in osteogenesis imperfecta.
复制标题
成骨不全症骨骼的病态解剖结构。
DOI：
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发表时间：
1981
影响因子：
4.2
通讯作者：
J. C. Lorenzo
中科院分区：
文献类型：
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作者：
P. Bullough;D. Davidson;J. C. Lorenzo
临床上，成骨不全的两个最显著的特征是矮小和骨脆性。放射学检查显示严重骨量减少伴或不伴多发畸形。与21例年龄匹配的对照组相比，16例髂骨活检标本的组织学检查显示，骨基质的数量显著减少，而骨细胞的数量却没有相关的减少，这表明胶原的产生存在数量上的不足。对46名受影响儿童的放射学研究和对两名儿童的尸检研究显示，骨化次级中心的骨化异常，同时有骨骺生长板断裂和碎裂的证据，导致典型的放射学外观，类似于一袋爆米花。在某些情况下，这些骨骺的异常可能导致肢体缩短。
Clinically, the two most prominent features of osteogenesis imperfecta are dwarfing and bone fragility. Radiologic examination reveals severe osteopenia with or without multiple deformities. Histological examination of samples obtained from 16 iliac crest biopsies compared with 21 age-matched controls revealed a significantly diminished amount of bone matrix without an associated decrease in the number of bone cells, suggesting a quantitative deficiency of collagen production. Radiologic studies of 46 affected children and autopsy studies of two children revealed abnormalities in the ossification of the secondary centers of ossification, together with evidence of fracture and fragmentation of the epiphyseal growth plates causing a characteristic radiologic appearance which may be likened to a bag of popcorn. These epiphyseal abnormalities may contribute to the limb shortening in some cases.