Variable osteoclast appearance in human infantile osteopetrosis
Variable osteoclast appearance in human infantile osteopetrosis
复制标题
人类婴儿石骨症中破骨细胞的变化
DOI：
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发表时间：
1988
影响因子：
4.2
通讯作者：
C. Anast
中科院分区：
文献类型：
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作者：
F. Shapiro;L. Key;C. Anast
摘要9例婴儿石骨症患者髂嵴干骺端骨的光镜和透射电镜（TEM）研究显示了不同的破骨细胞异常谱。所有骨均在治疗前活检获得。活检时的平均年龄为6个月，范围为1至12个月。破骨细胞的数量总是增加，细胞总是适当地定位在骨和软骨。破骨细胞数量、大小和成核程度从轻度到显著增加不等。在那些只有轻度到中度破骨细胞增加的患者中，骨髓具有接近正常的外观，具有良好的造血细胞补充。在破骨细胞显著增加（高骨密度状态）的患者中，只有少量造血细胞。光镜组织形态计量学记录破骨细胞覆盖的骨和软骨表面的百分比。最大破骨细胞存在区域的对照记录了5%的覆盖率。1例石骨症患者记录的值为4.8%，所有其他患者从7.6%升高至27.9%。定量电子显微镜检查显示，9例患者中有7例患者的皱褶边缘区复合体不存在或明显减少。在两个，但是，破骨细胞配置文件有丰富的皱褶边界透明区复合物。高骨密度骨髓患者的临床影响更严重。光镜和透射电镜研究骨髓活检初步评估骨硬化症建立了一个基线档案，可以提供预后信息，并允许更有意义的治疗随访。
SummaryA light and transmission electron microscopic (TEM) study of iliac crest metaphyseal bone from nine patients with infantile osteopetrosis demonstrates a variable spectrum of osteoclast abnormalities. All bone was obtained at biopsy before treatment. The average age at biopsy was 6 months with a range from 1 to 12 months. Osteoclast number was always increased and the cells were always appropriately positioned in relation to bone and cartilage. Osteoclast number, size, and nucleation varied from mildly to markedly increased. In those with only a mild-to-moderate osteoclast increase, the marrow had an otherwise near-normal appearance with a good complement of hematopoietic cells. In those with markedly increased osteoclasts (hyperosteoclastic state) there were only scanty collections of hematopoietic cells. Light microscopic histomorphometry documented the percentage of bone and cartilage surfaces covered by osteoclasts. Controls from areas of greatest osteoclast presence documented a 5% coverage. One osteopetrotic patient registered a 4.8% value with all others elevated from 7.6 to 27.9%. Quantitative electron microscopy showed the ruffled borderclear zone complex to be absent or markedly diminished in seven of the nine patients. In two, however, osteoclast profiles had abundant ruffled border-clear zone complexes. Patients with the hyperosteoclastic bone marrow were more severely affected clinically. Light and TEM studies of marrow biopsies in initial assessment of osteopetrosis establish a baseline profile, may provide prognostic information, and allow for more meaningful treatment follow-up.