颌骨骨纤维异常增殖症研究进展

Progresses in the study of fibrous dysplasia of the jaw bone

骨纤维异常增殖症(fibrous dysplasia,FD)是一种以纤维-骨性间质组织取代骨内部正常组织为特征的骨良性病变,在组织学水平表现为不同程度的骨化生。目前的研究表明,骨纤维异常增生是由于Gsα基因突变引起的。骨纤维异常增生可以只侵犯单一骨(单骨型),也可以侵犯多数骨(多骨型)。影像学检查以及病理检查是骨纤维异常增生症重要的诊断依据。对于骨纤维异常增生症的治疗仍存在争议,手术治疗难以确定统一的指征与手术范围,而保守的药物治疗疗效不尽人意。本文就本病的临床表现、诊断和治疗等方面的进展进行了综述。

[Summary] Fibrous dysplasia （ FD ） is a benign bone lesion. Histologically, the replacement of the normal substance of bone by fibro-osseous connective tissue is the most distinct character. Recent research shows that fibrous dysplasia is caused by a somatic activating mutation of the Gsα subunit of protein G. It may involve one bone （ monostotic ） or multiple bones （ polyostotic ）. Diagnosis relies on X-ray examination and pathology. The indications and extent of surgical therapy for craniofacial FD are controversial. Nonsurgical pharmacological treatments have been disappointing. This review presented the recent advances in clinical manifestations, diagnosis and treatment of FD of the jaw bones.