MORPHOLOGICAL-CHANGES IN GROWTH-PLATE CARTILAGE IN OSTEOGENESIS IMPERFECTA
MORPHOLOGICAL-CHANGES IN GROWTH-PLATE CARTILAGE IN OSTEOGENESIS IMPERFECTA
复制标题
DOI：
10.1302/0301-620x.72b3.2187879
复制
发表时间：
1990-05-01
影响因子：
--
通讯作者：
FALCIGLIA, F
中科院分区：
文献类型：
--
作者：
SANGUINETTI, C;GRECO, F;FALCIGLIA, F
我们从四位成骨不全的病人身上取得生长板软骨标本。光学显微镜显示组织结构的变化和软骨细胞和基质的形态学变化，特别是在肥大区。在软骨的主要矿化区的钙化过程中有变化。我们还发现了组织化学变化的基质糖胺聚糖（GAG）的生理矿化被干扰的区域和小梁中断和矿化不良。除了胶原蛋白中已知的分子缺陷之外，GAG和非胶原蛋白的变化是疾病发病机制中的重要因素。
We obtained specimens of growth-plate cartilage from four patients with osteogenesis imperfecta. Light microscopy showed structural changes in the tissue and morphological changes in chondrocytes and matrix, particularly in the hypertrophic zone. There were changes in the process of calcification in the primary mineralisation zone of the cartilage. We also found histochemical changes in the matrix glycosaminoglycans (GAGs) in the zones where physiological mineralisation was disturbed and where the trabeculae were interrupted and poorly mineralised. In addition to the known molecular defects in collagen, changes in GAGs and non-collagenous proteins are important factors in the pathogenesis of the disease.