纤维结构不良的致病机制和治疗研究进展  

Research Progress of Pathogenesis and Treatment of Fibrous Dysplasia

作　　者：侯振兴 段星宇 廖文胜 刘明阳 高延征 HOU Zhenxing;DUAN Xingyu;LIAO Wensheng;LIU Mingyang;GAO Yanzheng(Department of Spinal Surgery,Henan Provincial People′s Hospital,Zhengzhou 450003,China;Department of Orthopedics,the First People′s Hospital of Zhengzhou,Zhengzhou 450004,China)

机构地区：[1]河南大学人民医院脊柱外科,郑州450003 [2]郑州市第一人民医院骨科,郑州450004

出　　处：《医学综述》2024年第9期1098-1103,共6页Medical Recapitulate

基　　金：河南省博士后科研项目(202103115);河南省医学科技攻关计划项目(SBGJ202103019)。

摘　　要：纤维结构不良(FD)是一种由基因突变导致的罕见良性骨肿瘤,主要由鸟嘌呤核苷酸结合蛋白α刺激活性多肽基因突变导致骨骼矿化缺陷、局部破骨细胞增多,从而引起正常骨骼被纤维组织侵蚀。FD可累及各部位骨骼,尚缺乏针对性的分类系统,且难以制订标准术式,目前一般采用髓内钉、椎体切除内固定等手术方式治疗。FD的药物治疗常使用双膦酸盐以缓解骨痛,降低骨折风险。核因子κB受体活化因子配体(RANKL)抑制剂地诺单抗通过抑制FD患者体内RANKL的过量表达阻止病变进展,而生长激素受体拮抗剂培维索孟可降低生长激素水平,故用于伴有内分泌疾病FD患者的治疗。Fibrous dysplasia(FD)is a rare benign bone neoplasms caused by gene mutations.It is mainly due to the guanine nucleotide-binding proteinα-stimulating activity polypeptide gene mutation that leads to defective bone mineralization and an increase in local osteoclasts,causing normal bones to be eroded by fibrous tissue.FD can involve bones at any position,and there is a lack of a specific classification system and it is difficult to formulate standard surgical procedures.Currently,intramedullary nailing,vertebral body resection and internal fixation are generally used for surgical treatment.Diphosphonates are commonly used as drug treatments to relieve bone pain and reduce the risk of fractures of FD.The receptor activator of nuclear factor-κB ligand(RANKL)inhibitor denosumab targets the overexpression of RANKL in FD patients to prevent disease progression,and the growth hormone receptor antagonist pegvisomant reduces growth hormone levels to treat FD patients with endocrine diseases.

关 键 词：骨肿瘤 纤维结构不良 核因子ΚB受体活化因子配体 地诺单抗 

分 类 号：R738.1[医药卫生—肿瘤]