MORPHOLOGICAL FEATURES OF BONE IN HUMAN OSTEOPETROSIS
MORPHOLOGICAL FEATURES OF BONE IN HUMAN OSTEOPETROSIS
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DOI：
10.1016/8756-3282(91)90030-m
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发表时间：
1991-01-01
期刊：
影响因子：
4.1
通讯作者：
SCHERFT, JP
中科院分区：
文献类型：
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作者：
HELFRICH, MH;ARONSON, DC;SCHERFT, JP
从6例恶性石骨症患者，1例良性石骨症患者和2例对照组获得的骨小梁活检进行了光镜和电镜检查。骨硬化性破骨细胞几乎没有主动参与骨吸收的迹象。大多数牢房没有皱褶的边界和透明的区域。所有患者均存在大量破骨细胞。所有恶性石骨症患者的成骨细胞、骨衬里细胞和骨髓基质细胞数量均极低。在6名患者中，观察到无定形有机材料的矿化层，其缺乏胶原纤维，覆盖大面积的骨或软骨基质。我们认为这一层代表了病理性钙化，随后有机物质在其上积累。在骨硬化症骨的异常进行了讨论，根据这种疾病的发病机制。
Trabecular bone biopsies obtained from six patients with malignant osteopetrosis, one patient with benign osteopetrosis, and two controls were examined by light and electron microscopy. Osteopetrotic osteoclasts showed little to no signs of active involvement in bone resorption. Ruffled borders and clear zones were absent in most cells. In all patients there were large numbers of osteoclasts. Numbers of osteoblasts, bone lining cells, and bone marrow stromal cells were extremely low in all patients with malignant osteopetrosis. In six of the patients a mineralized layer of amorphous organic material lacking collagen fibrils was seen covering large areas of the bone or cartilage matrix. We suggest that this layer represents a pathological calcification on which subsequently organic material has accumulated. The abnormalities in osteopetrotic bone are discussed in the light of the pathogenesis of this disease.