Ralphs J R, Stamp T C, Dopping-Hepenstal P J, Ali S Y
Institute of Orthopaedics (University of London), Royal National Orthopaedic Hospital, Stanmore, Middlesex, England.
Bone. 1989;10(4):243-9. doi: 10.1016/8756-3282(89)90060-4.
The osteoid of a patient with Fibrogenesis Imperfecta Ossium is described. Three iliac crest biopsies were taken; firstly before treatment, secondly after calcitriol therapy and finally after successful treatment with melphalan and prednisolone. In the pretreatment biopsy the osteoid was greatly enlarged, showed complete absence of the birefringence characteristic of oriented collagen fibers, and at ultrastructural level was shown to be composed of abnormal collagen fibrils. The fibrils were often curved and were extremely variable in thickness. Calcification within the osteoid took the form of calcospherites and spread of calcification from these to collagen fibrils was greatly delayed. In the second biopsy two aspects of osteoid ultrastructure were noted; some samples resembled the first biopsy, but others had a different organization. The osteoid of these samples had two regions: an inner region containing abnormal collagen fibrils and an outer region composed of moderately electron-dense amorphous material. The osteoblasts associated with this region were clearly highly biosynthetically active. The third biopsy, after treatment with Melphalan and prednisolone, showed a reversion to more normal bone ultrastructure with uniform, oriented collagen fibrils and prompt mineralization resulting in narrow osteoid seams. Remnants of the original abnormal osteoid were present in the marrow space as calcified debris. Reasons for the success of this therapeutic regime are unclear; however, some speculation is made as to the possible roles of the cytotoxic drug and the glucocorticoid in the regression of this condition.
描述了一名骨纤维发育不全患者的类骨质情况。进行了三次髂嵴活检；第一次在治疗前，第二次在骨化三醇治疗后，最后一次在美法仑和泼尼松龙成功治疗后。在治疗前的活检中，类骨质显著增大，完全缺乏定向胶原纤维的双折射特征，在超微结构水平显示由异常胶原原纤维组成。这些原纤维常常弯曲，厚度变化极大。类骨质内的钙化呈钙球形式，从这些钙球向胶原原纤维的钙化扩散大大延迟。在第二次活检中，注意到类骨质超微结构的两个方面；一些样本类似于第一次活检，但其他样本有不同的组织结构。这些样本的类骨质有两个区域：一个内部区域含有异常胶原原纤维，一个外部区域由中等电子密度的无定形物质组成。与该区域相关的成骨细胞显然具有高度的生物合成活性。第三次活检是在美法仑和泼尼松龙治疗后，显示恢复到更正常的骨超微结构，有均匀、定向的胶原原纤维和迅速矿化，导致类骨质缝变窄。原始异常类骨质的残余物以钙化碎片的形式存在于骨髓腔中。这种治疗方案成功的原因尚不清楚；然而，对细胞毒性药物和糖皮质激素在这种疾病消退中的可能作用进行了一些推测。
