Kuo T T, Davis C P
Hum Pathol. 1981 Apr;12(4):376-9. doi: 10.1016/s0046-8177(81)80149-9.
Scanning electron microscopic investigation was conducted with bone in a case of osteopetrosis by comparing it with normal cortical bone. The fractured surface of the osteopetrotic bone was devoid of the orderly constructed lamellar haversian system seen in normal bone. It was occupied by a massive smooth cartilaginous matrix containing scattered rough areas of abnormal ossification. Tortuous channels were randomly distributed and corresponded to the abortive marrow spaces. The presence of many irregular fracture lines reflected the fragility of the bone. The findings support the concept of defective remodeling in the pathogenesis of osteopetrosis and provide a further explanation for the abnormal fragility of bone in osteopetrosis.
通过将骨石化症患者的骨骼与正常皮质骨进行比较，进行了扫描电子显微镜研究。骨石化症骨骼的骨折表面没有正常骨骼中有序构建的板层哈弗斯系统。它被大量光滑的软骨基质占据，其中含有散在的异常骨化粗糙区域。曲折的通道随机分布，对应于发育不全的骨髓腔隙。许多不规则骨折线的存在反映了骨骼的脆弱性。这些发现支持了骨石化症发病机制中重塑缺陷的概念，并为骨石化症中骨骼异常脆弱提供了进一步的解释。
