先天性胫骨假关节中肌纤维母细胞的表达研究

Expression of myofibroblast in congenital pseudarthrosis of the tibia

目的深入了解先天性胫骨假关节(CPT)的病理变化、细胞类型及其病变来源。方法(1)取5例先天胫骨假关节(CPT)标本中不同部位的组织20例,经固定、切片染色后置于透射电镜下进行观察。(2)同时采用免疫组化及免疫荧光方法对24例CPT病人的25个病变骨膜标本中α-平滑肌肌动蛋白(α-SMactin)、波纹蛋白(vimentin)、结蛋白(desmin)进行检测。以正常骨膜(10例),创伤性假关节(TP)骨膜(20例)和纤维瘤病组织(10例)作为对照。结果(1)断端间组织及病变骨膜组织性质完全一样,均为致密纤维结缔组织,细胞成分多,主要纤维母细胞、肌纤维母细胞及少量未分化细胞。移行部位骨膜组织结构与病变处骨膜组织结构相似。(2)所有标本均为波纹蛋白染色阳性,结蛋白染色阴性,20例先天性胫骨假关节骨膜和8例纤维瘤病病变组织中α-平滑肌肌动蛋白染色阳性,两者无显著性相差(P>0.05),正常骨膜及创伤性假关节(TP)骨膜中α-平滑肌肌动蛋白染色阴性,与先天性胫骨假关节骨膜和纤维瘤病病变组织有显著性相差(P<0.01)。结论(1)先天胫骨假关节(CPT)是非神经起源的、而是起源于骨膜的一种细胞增生活跃的纤维增生性变;(2)肌纤维母细胞是先天胫骨假关节发病中关键的病理因素之一。

Objective To investigate the pathological changes and kinds of cell and origin of congenital pseudarthrosis of tibia(CPT).Method (1) 20 different specimens were taken from 5 CPT patients .By electron microscopy all specimens were studied after fixation and dying.(2) 25 specimens were taken from 21 CPT patients .Streptoavidin Peroxidase were applied to determine the expression of level of α SM actin,Vimentin and Desmin .The control specimens were taken from normal periosteum(10 cases),fibromatiosis(10 cases) and traumatic pseudarthrosis(20 cases).Result (1)The soft tissue interposed between the CPT bone ends had the same pathological change as CPT periosteum.Both of them were proliferated dense connective tissues in which there were a large number of cellular components and collagen fibers, including plenty of fibroblast myofibroblast and some indifferential cells.The pathological change of the periosteum between the normal periosteum and CPT periosteum was similar to the CPT periosteum.(2) All specimens were positive for Vimentin and negative for Desmin. 20 specimens of CPT and 8 specimens of fibromatiosis were positive for α SM actin ,there was not significant difference of expression level of α SM actin between the periosteum of CPT and fibromatosis(P >0.05). The specimens of normal periosteum and traumatic pseudarthrosis were negative for α SM actin, The expression of α SM actin in fibromatosis and the periosteum of CPT was higher than that in normal periosteum and traumatic pseudarthrosis(P< 0.01).Conclusion (1)CPT is a non neuro original but periosteum original fibrosis which had a strong activity of cellular proliferation and corrosion.(2) Myofibroblast appeared to be one of the most important factors in the pathogenesis of congenital pseudarthrosis of tibia.