|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|
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.
|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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