先天性胫骨假关节的病因病理学研究  

Etiological and Pathological Investigations of Congenital Pseudarthrosis of the Tibia

作　　者：雷伟[1] 黄耀添[1] 王剑波[1] 吕荣[1] 杨光[1] 

机构地区：[1]第四军医大学西京医院全军创伤骨科研究所

出　　处：《中华骨科杂志》1998年第11期649-653,I001,共6页Chinese Journal of Orthopaedics

摘　　要：目的：先天性胫骨假关节（ＣＰＴ）的病因及病理仍不完全清楚，临床治疗困难，复发率高，是骨科领域一个十分棘手的问题。本研究旨在探讨其发病原因、病理特点及可能的发病机制，为更合理的临床治疗提供理论依据。方法：２１例ＣＰＴ中不同部位的６３个标本，采用大体、显微镜、电镜观察，并首次采用免疫组化技术。抗体分别为抗：（１）神经丝蛋白（ＮＦ－２００）、Ｓ－１００蛋白，（２）Ⅰ、Ⅲ型胶原蛋白，（３）增殖细胞核抗原（ＰＣＮＡ），（４）转化生长因子β１（ＴＧＦ－β１），（５）雌激素受体（ＥＲ）。选用神经纤维瘤病和创伤性假关节为对照组。ＳＡＢＣ法染色判定结果。结果：ＣＰＴ骨膜及其周围有范围广泛、边界不清的致密纤维结缔组织增生，并对胫骨形成包裹、压迫和侵蚀。显微镜及电镜下见大量高分化纤维母细胞和胶原纤维增生，未见异常神经成分。ＮＦ－２００、Ｓ－１００在ＣＰＴ中阳性表达率极低，分别为３．２％和４．８％，而在神经纤维瘤病中分别为８５．２％和８８．９％，两者差异有非常显著性意义（Ｐ＜０．０１）。与创伤性假关节相比，ＣＰＴ软组织病变组织中的Ⅲ型胶原比例上升，ＰＣＮＡ阳性表达率高，而ＴＧＦ－β１和ＥＲ的阳性表达率则很低，其差异均有非常显著性意?Objective: To investigate the etiology and pathology of congenital pseudarthrosis of the tibia(CPT) and to provide a scientific base for a reasonable protocol of treatment. Methods: Sixty three specimens were taken from 21 CPT patients. This research project was undertaken by means of macroscopy, microscopy, electronic microscopy and immunohistochemistry. The antibodies used for the immunohistochemical studies, were produced against1) NF- 200 or S- 100 protein, 2) Type Ⅰ or Ⅲ collagen, 3) PCNA, 4) TGF- β 1, 5) Estrogen receptor. The control specimens were taken from neurofibromatosis and traumatic pseudarthrosis. SABC staining was used. Results: A large number of proliferated dense connective tissues encapsuled, compressed and eroded the tibia, but had no clear margin at the lesions of CPT and its surrounding soft tissues, a large number of fibroblast cells and collagen fibers existed in the CPT periosteum and the interposed soft tissues between the bone ends. However, abnormal neurofibers was not found under the light and electronic microscopes. Resorption and necrosis happened in the bone ends at the CPT lesion and low positive expression of NF- 200(3.2%) and S- 100(4.8%) in the CPT lesion were seen, but high expression of NF- 200(85.2%) and S- 100(88.9%) in the lesion of neurofibromatosis were found. The soft tissues in CPT had a higher ratio of type Ⅲ to Ⅰ collagens and positive expression of PCNA,lower ratio of positive expression of TGF- β 1 and estrogen receptor than that in the traumatic pseudarthrosis. Conclusion: CPT is a non neuro originated disease and has no direct relation with neurofibromatosis. CPT has a pathology characteristic in the periosteum rather than in the bone. The pathological fracture or non union of CPT is caused by the fibromatosis, which has a strong activity of cellular proliferation and corrosion. CPT has a pathological property similar to a neoplasm. CPT is supposed to be associated with the lower amount of ER, lower synthesis and secretion of TGF- β 1 and the

关 键 词：假关节 胫骨假关节 病理学 先天性 CPT 

分 类 号：R681.802[医药卫生—骨科学]