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骨自溶症的临床特点与治疗——附六例病例分析
廖锋
2016-05-26
导师田伟
专业外科学
授予单位北京大学
授予地点北京大学第四临床医学院
学位博士
关键词骨自溶症 诊断 治疗 双磷酸盐
其他题名Gorham’s disease:Six cases study
分类号R681
摘要

目的:骨自溶症为一种原因不明的罕见病,以局部骨组织逐渐被富含薄壁脉管的纤维结缔组织替代为特征,目前全世界仅报道200余例。本研究报道分析就诊于积水潭医院骨肿瘤科的骨自溶症患者的临床特点和治疗情况。

资料及方法:纳入于2000年至2015年就诊于北京积水潭医院骨肿瘤科的所有骨自溶症病例。回顾性分析患者的临床、影像、病理、治疗及随访资料。

结果:共有6例患者,于2003年至2015年就诊于我科。6例患者男女比例为4:2。中位发病年龄为23.5岁(14-33岁)。发病部位为:肩胛带周围3例、骨盆周围3例。4例患者因局部疼痛就诊、另两例患者则在外伤后行影像学检查时偶然发现病灶。影像学表现均为进行性的局部骨组织溶解消失,病灶与正常骨边界清楚,不伴软组织肿块;镜下可见丰富的薄壁脉管样结构,缺乏细胞异型性。2例患者未接受任何治疗,其中1例失访,另一例随访3年,疾病不断进展。2例患者就诊于我院前,在外院行刮除植骨。4例患者接受双磷酸盐治疗,其中治疗时间超过1年的1例患者,病变已稳定;其余3例患者目前治疗尚未结束,但可见病变进展减缓。

结论:骨自溶症为罕见病,诊断主要依靠影像和病理。双磷酸盐治疗可能有助于促进病灶稳定。

英文摘要

Objective: Gorham’s disease is a rare disorder of unknown etiology acterized histologically by the proliferation of thin-wall vessels in bone and by the replacement of bone with fibrous tissue. There are just about 200 cases reported in the literature till date. Here, we present the cases of Gorham’s disease diagnosed and managed in our department.

Materials and methods: All patients diagnosed as Gorham’s disease in Beijing Jishuitan Hospital from 2000 to 2015 were included. Their clinical features, radiographic findings, pathology, treatment, and outcome were collected by retrospective medical record review.

Results: Six patients diagnosed and/ or treated for Gorham’s disease in the department of orthopedic oncology surgery of Beijing Jishuitan Hospital from 2003 to 2015. The median age of six patients (4 male and 2 female) at initial visit was 23.5 years (range 14-33 years). The reasons for referral to the orthopedist were local pain in four patients and asymptomatic skeletal lesion found after trauma in two other patients. Bones of shoulder and hip were the major location affected with acteristic lesions: progressive and non-expansile local osseous resorption. Biopsies of all the bony lesions were reported: containing numerous thin-wall vessels and absence of cellular atypia. Two patients did not receive any treatment, one of them lost to follow-up, the other was living with progressive disease 3 years following initial visit. Before referred to our department, two patients received curettage and bone grafting. Four patients received treatment with bisphosphonates, this lead to stabilization of bony lesions and healing of pathological fracture in one patient who treated for more than one year, while the other three patients was still under treatment at last follow-up.

Conclusion: The diagnosis Gorham's disease is mainly based on radiographical and histological data. Bisphosphonates may be effective in arresting the disease.

语种中文
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文献类型学位论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/124760
专题北京大学第四临床医学院
作者单位北京大学第四临床医学院
推荐引用方式
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廖锋. 骨自溶症的临床特点与治疗——附六例病例分析[D]. 北京大学第四临床医学院. 北京大学,2016.
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