Vignery A
Yale University School of Medicine, Departments of Orthopaedics and Cell Biology, New Haven, CT 06510, USA.
Connect Tissue Res. 1995;31(4):275-8. doi: 10.3109/03008209509010822.
Iliac crest bone biopsies from nine children (6-15 years old) with osteogenesis imperfecta tarda (O.I.) have been studied by bone histomorphometry after double fluorescent labeling with tetracycline and compared to five unlabeled biopsies from normal children in the same age group. The results indicate that children with O.I. have a low trabecular bone volume associated with an increased bone turnover rate. Bone formation is increased at the tissue level despite a decrease in the activity of individual osteoblasts. The original defects in O.I. seem to be due to the altered rate of matrix synthesis by osteoblasts. It is, however, compensated by an increase in the number of these cells. These results suggest that these children were not losing bone at the time of the biopsy, which fits with the clinical stability of O.I. with age. Our study therefore suggests that the osteopenia observed in O.I. is most likely due to an inability to accumulate bone during growth, as normal children do, rather than to a progressive net loss of bone.
对9名患有迟发性成骨不全症（O.I.）的儿童（6至15岁）的髂嵴骨活检标本进行了四环素双荧光标记后的骨组织形态计量学研究，并与来自同一年龄组正常儿童的5份未标记活检标本进行了比较。结果表明，患有O.I.的儿童小梁骨体积较低，同时骨转换率增加。尽管单个成骨细胞的活性降低，但在组织水平上骨形成仍增加。O.I.的原始缺陷似乎是由于成骨细胞基质合成速率的改变。然而，这通过这些细胞数量的增加得到了补偿。这些结果表明，这些儿童在活检时并未骨质流失，这与O.I.随年龄增长的临床稳定性相符。因此，我们的研究表明，在O.I.中观察到的骨质减少最可能是由于无法像正常儿童那样在生长过程中积累骨质，而不是由于骨质的渐进性净流失。
