摘要
目的 了解先天性胫骨假关节 (CPT)的病理变化、病变来源及可能的病因。方法 采用电镜、免疫组织化学及细胞培养等方法对 2 5例CPT病变骨膜标本中胶原、α 平滑肌肌动蛋白、骨形态形成蛋白 (BMP)等细胞因子及CPT患者染色体核型进行检测。以创伤性假关节 (TP)和纤维瘤病组织标本为对照。结果 (1)电镜研究发现CPT断端间组织及病变骨膜组织均为致密纤维结缔组织 ,细胞成分多 ,主要为纤维母细胞、肌纤维母细胞等。 (2 )组织化学研究发现CPT骨膜及纤维瘤病病变组织中以Ⅲ型胶原为主 ,与正常骨膜和TP骨膜差异有显著意义 (P <0 0 5 )。 (3)免疫组织化学及免疫荧光化学研究发现所有标本波纹蛋白染色阳性 ,结蛋白染色阴性 ,2 0例CPT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 ,两者差异无显著意义 (P >0 0 5 ) ,但与正常骨膜及TP骨膜间差异有显著意义 (P <0 0 1)。另外CPT骨膜和纤维瘤病病变组织中未见BMP阳性染色 ,与正常骨膜和TP骨膜相比差异有显著意义 (P <0 0 5 ) ;CPT骨膜和纤维瘤病病变组织中均有部分标本中白细胞介素1、白细胞介素 6、肿瘤坏死因子 α,碱性成纤维细胞生长因子为阳性染色 ,与正常骨膜及TP骨膜相比差异有显著意义 (P <0 0 5 )。 (4)细胞培养研究发现 11例CPT患者染色?
Objective To investigate the histopathology, origin, and etiology of congenital pseudarthrosis (CPT). Methods Specimens of periosteum from 28 CPT cases, 20 cases of traumatic pseudarthrosis (TP), 10 cases of fibromatosis, and 10 normal controls were examined. The pathological changes were observed by electron microscopy and confocal microscopy. The expressio of α smooth muscle (SM) actin, vimentin, desmin, bone morphogenic protein (BMP), interleukin (IL) 1, IL 6, tumor necrosis factor α (TNF α) and base fibroblast growth factor (b FGF) were detected by histochemistry and immunofluorescence chemical staining. Chromsomal karyotype was examined among 11 cases of CPT. Results (1) Electron microscopy showed that the periosteum and soft tissue between the broken ends in CPT were all dense fibrous connective tissue abundant in cells, including fibroblasts, myofibroblasts, etc. (2) The chief collagen element was type I collagen in normal periosteum and was type III collagen in periosteum of patients with CPT and fibromatosis ( P <0.025). (3) Vimentin was positive and desmin was negative in all specimens. The expression of α SM actin was higher in specimens from CPT and fibromatosis than in specimens from normal control and TP ( P <0.01). The expression of BMP was higher in normal periosteum and TP than in the periosteum of CPT and fibromatosis ( P <0.05). The expression of IL 1, IL 6, TNF α, b FGF was higher in the periosteum of CPT and TP ( P <0.01). (4) The chromosomal karyotype of all CPT patients was noemal 46XY or 46XX. Conclusion (1) Neurofibromatosis is probably not the etiological factor of CPT. (2) CPT is a kind of invasive fibromatosis located in periosteum. (3) Abnormal expression of many kinds of cytokine and high expression of type III collagen play an important role in the pathogenesis of CPT. (4) The main pathologiy of CPT is hyperplasia of fibroblasts, thus causing thickening of periosteum, contractible circinate coarctation, and compression of tibia and surrounding tissues. (5) The chromosomal karyotype of patients with CPT is normal.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2002年第7期487-491,共5页
National Medical Journal of China
