股骨近端纤维结构不良的分型及治疗进展

Current treatment and classification for fibrous dysplasia of proximal femur

纤维结构不良是一种罕见的起源于骨的良性病变,是由于正常骨和骨髓被替换为纤维骨组织。病因主要与鸟苷酸结合蛋白活性α活性刺激肽基因突变有关。其中以股骨近端病变最为常见。临床表现主要有疼痛、跛行、畸形和病理性骨折。为了便于交流及规范化治疗,各国学者分别制定了四种股骨近端纤维结构不良的分型方式及对应的治疗策略,文中就股骨近端纤维结构不良的各种分型及治疗研究进展作一综述。

Fibrous dysplasia is a benign skeletal lesion, in which normal bone and bone marrow are replaced by fibro-osseous tissue. The pathogenesis of fibrous dysplasia may be related to activating of GNAS gene. The proximal femur is the most commonly affected areas. The main clinical symptoms are pain, limp, deformities and pathological fractures. In order to facilitate the academic exchange and standardized treatment, scholars have developed four classifications for fibrous dysplasia of the proximal femur and corresponding treatment strategies. This article is aim to review the progress in the treatment and classification of fibrous dysplasia.