Nogami H, Ono Y, Katoh R, Oohira A
Department of Orthopaedic Surgery, Central Hospital, Aichi, Japan.
Clin Orthop Relat Res. 1993 Jul(292):358-65.
Periosteum stripped from fragments of diaphyseal long bones from osteogenesis imperfecta (OI) patients was examined both in vivo and in vitro. The OI periosteum was thickened and characteristic finding was a defective microvascular system. The vascular walls of arterioles and capillaries were thick and the lumina of many of these vessels were narrowed or occluded by hypertrophic endothelial cells and multiplication of smooth muscle cell layers. Circulatory deficiency in the OI periosteum might be regarded as one of the causes of the slender, osteopenic diaphyseal bone characteristic of the disorder. A higher proliferative activity was observed in cultured OI periosteal cells, but the cytoplasmic structure showed increased myelinoid bodies and smooth endoplasmic reticulum and decreased lysosomes and rough endoplasmic reticulum. These findings may be related to the abnormal metabolic activity of OI periosteal cells.
对取自成骨不全（OI）患者骨干长骨碎片的骨膜进行了体内和体外检查。OI骨膜增厚，其特征性发现是微血管系统存在缺陷。小动脉和毛细血管的血管壁增厚，许多这些血管的管腔被肥大的内皮细胞和平滑肌细胞层增生所狭窄或阻塞。OI骨膜中的循环缺陷可能被视为该疾病特征性的细长、骨质减少的骨干骨的原因之一。在培养的OI骨膜细胞中观察到较高的增殖活性，但细胞质结构显示髓样小体和平滑内质网增加，溶酶体和粗面内质网减少。这些发现可能与OI骨膜细胞的异常代谢活动有关。
