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1、,骨纤维结构不良Fibrous dysplasia许树柴郭海威黄泽鑫刘岩广州中医药大学附属广东省中医院二沙岛骨科,FR骨纤维结构不良骨纤维结构不良,又称骨纤维异常增殖症,是一种以异常增生的纤维骨组织或不成熟的编织骨取代正常的骨组织为特点的良性病变。最早由 Lichtenstein和aft1942年提出,可分为单骨型、多骨型及McCune-Albright综合征(MAS)。McCune-Albright综合征(MAS)骨纤维异常增殖合并骨骼外的病变。,The epidemiology of FD MAS is poorly understood. Mild,流行病学udies, and many
2、 patien.s likely go undiagnosed. In addi-tion, nbro-Dssecus lesions are pooriy character ized, whichmary reslt in an wvendignasis of monostotic FD in patientswith isolated bony lesions I. Additionally, patients whoareealuated for polyostotic and endocrine discase, making itficult ta determine the re
3、lative prevalence af these subtypes. A meta-analysis of 18 serics including a total of 487骨纤维结构不良的国内发病率为1030/100万,占骨肿瘤样病变的首位,发病人群以儿童及青少年多见,通常不易恶变。A meta-analysis of 18 series including a total of 487 patients diagnosed with craniofacial FDreported that 56% had monostotic disease, 47% had polyostotic
4、 disease, and 7% had MAS 2. It isunclear whether these patients were screened for subclinical endocrine disease, calling intoques x0002 tion the generalizability of these fndings,FR流行病学国外报道发病率约为150001000好发于儿童及青年,无性别差异,占所有良性骨病变的5%;单骨型最为常见,约占骨纤维结构不良的7580%单骨型多发生在长骨,常见于股骨和胫骨;多骨型多发生在颅面骨、肋骨、骨盆和长骨;也可发生于手及脊
5、柱等骨纤维结构不良最常见的累及部位是股骨。,FRMAS骨外病变表现:1咖啡斑色素沉着:锯齿状、边界不规则、一般不过身体中线2性早熟:女孩复发性卵巢囊肿、男孩睾丸病变3甲状腺病变或甲亢4生长激素过量5新生儿皮质醇增多症6低磷血症,FR咖啡斑色素沉着锯齿状边界不规则一般不过身体中线refering alme the nid ine ufrer hilrenl Izaerestrerredleg tmn ver,FRA:卵巢囊肿睾丸增大C:甲状腺弥浸性结节D:垂体大腺瘤,FR发病机制:1基因突变:与20号染色体上GNAS1基因的错义突变有关,突变激活后会导致纤维组织的异常增殖。2染色体变异:2号染色体三体、核型异常。3.内分泌异常:病变组织的成骨细胞内甲状旁腺激素相关蛋白PTHP的表达水平明显增高。4骨发育异常:骨祖母细胞向成骨细胞转化存在着障碍。,FR临床特点早期可无症状;随着病情发展,出现局部疼痛及深压痛、功能障碍、病理性骨折、畸形病变累及骨骺时,常由于骨骺端病变跨越骺板而致畸形,长管状骨畸形通常是因为骨折畸形愈合或变软的骨骼受到外力牵拉所致,股骨牧羊拐样畸形颈干角变小股骨弯曲变形患肢短缩,
