关于戈勒姆病的研究进展

doi:10.3969/j.issn.1674-490X.2023.05.005

摘要/Abstract

摘要： 戈勒姆病是一种罕见的综合征,该综合征在Hardegger分类中被认为是Ⅳ型骨溶解,病因及发病机制目前尚不清楚。戈勒姆病的骨科临床特征取决于受影响的骨骼,其特征是影响一块或多块骨头的大量骨溶解,并以骨淋巴管替代,可能危及生命。该病的诊断极具挑战性,是一种排除性诊断,除此之外,影像学检查也是作出诊断的必要条件。该病以综合治疗为主,西罗莫司可以抑制细胞增殖和血管生成;双膦酸盐具有抗溶骨活性,抑制破骨细胞导致的骨溶解;放射治疗可能通过抑制内皮细胞增殖来阻止骨溶解的进展;手术主要用于治疗并发症。由于该病合并乳糜胸等并发症时常常预后不良,因此进一步研究是十分有必要的,以此来获得对于此病更深入的见解,指导临床最合适的治疗策略。

关键词: 戈勒姆病, 临床表现, 诊断, 治疗

Abstract: Gorham disease is a rare syndrome, which is considered as type Ⅳ osteolysis in hardegger classification. The etiology and pathogenesis are unclear. The orthopaedic clinical features of Gorham disease depend on the affected bones. It is characterized by massive osteolysis affecting one or more bones and replaced by bone lymphatic vessels, which may be life-threatening. The diagnosis of the disease is very challenging and is an exclusive diagnosis. In addition, imaging examination is also a necessary condition for diagnosis. The disease is mainly treated by comprehensive treatment, sirolimus can inhibit cell proliferation and angiogenesis; bisphosphonates has anti-osteolytic activity and inhibits osteolysis caused by osteoclasts; radiotherapy may prevent the progression of osteolysis by inhibiting the proliferation of endothelial cells; surgery is mainly used to treat complications. Because the prognosis of chylothorax and other complications is often poor, further research is very necessary to obtain more in-depth insights into the disease and guide the most appropriate clinical treatment strategy.

Key words: Gorham disease, clinical manifestation, diagnosis, treatment

中图分类号:

张印恩,于欢欢,王键龙,胡斌,曾诚,林文哲,徐瑞轩,焦建宝. 关于戈勒姆病的研究进展[J]. 医学研究与教育, 2023, 40(5): 29-37.

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