中文摘要：

目的探讨骨纤维结构不良症（FD）的临床特征。方法回顾性分析我院2003—2007年收治的113例FD患者的临床资料。结果年龄为2～75岁的113例患者中，男性50例，女性63例，平均发病年龄（26．0±16．3）岁，其中31岁以下占62．8％。主诉局部疼痛者79例，肿胀畸形者42例，活动受限和功能障碍者19例，发生病理性骨折21例；其中单骨型91例（79．6％），共累及全身骨骼161块，以股骨最常受累；46例（40．7％）患者血碱性磷酸酶（ALP）升高，中位数为94IU／L，3例患者出现低血磷；X线表现为病灶部位呈毛玻璃样变、丝瓜瓤及囊性膨胀性改变等；骨核素显像（ECT）表现为特征性放射性浓聚。结论骨纤维结构不良好发于11～30岁之间，常单骨发病，股骨最易受累，因此，临床上对于局部出现疼痛、肿胀畸形等症状的青少年，应及早行X线和血ALP等检查，以减少误诊。

英文摘要：

Objective To analyze the clinical characteristic of fibrous dysplasia（FD）. Methods The clinical data of 113 patients with FD treated in the Sixth People＇s Hospital, Shanghai Jiaotong University from 2003 to 2007 were collected and analyzed retrospectively. Results In 113 patients, 63 were female, and 50 were male. The onset mean age was （26.0 ± 16.3） years（ranging from 2 to 75） and under the age of 31 accounted for 62.8% . The patients had various clinical manifestations including local pain （79 cases）, swelling or deformity （42 cases）, functional disturbance （19 cases） and pathologic fracture （21 cases）. Ninety-one cases presented with monostotic FD accounting for 79.6%, and involved 161 bones, especially the femoral bones （58.4%）. There were 46 patients who had higher serum ALP, and the median value was 94 IU/L. Three patients had hypophosphatemia. In typical cases X ray showed ＂ground-glass＂ pattern, cystic type or sponge ground-flesh type. Twenty-seven cases were performed ECT and showed radioactive accumulation in the local lesions. Conclusion Fibrous dysplasia is predominantly found between 11 and 30 years old. The monostotic FD is much more common and the most commonly affected site is the femur. The patients often complain of local pain or swelling. Therefore, adolescents with local pain or swelling should be alert fibrous dysplasia. To reduce misdiagnosis, X-ray and serum ALP should be performed as a routine screening procedure as soon as possible.