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戈勒姆病:一种淋巴管生成性骨病?

Gorham's disease: an osseous disease of lymphangiogenesis?

作者信息

Radhakrishnan Kavita, Rockson Stanley G

机构信息

Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA.

出版信息

Ann N Y Acad Sci. 2008;1131:203-5. doi: 10.1196/annals.1413.022.

Abstract

Gorham's disease, also known as massive osteolysis, Gorham-Stout disease, vanishing bone disease, or, phantom bone disease is a rare disorder of the musculoskeletal system. The disease is characterized by osteolysis in bony segments, with localized proliferation of lymphatic channels. The presence of abundant, leaky systemic lymphatic vessels is often accompanied by chylous ascites. There is no standardized treatment available for Gorham's disease, and its molecular mechanisms remain unclear. Future strategies for understanding Gorham's disease should emphasize its apparent identity as a disease of disordered lymphangiogenesis. Breakthroughs in lymphatic research have identified several lymphangiogenic pathways that may play a relevant role in Gorham's disease.

摘要

戈勒姆病,也称为大块骨质溶解症、戈勒姆-斯托特病、骨质消失症或幻影骨病,是一种罕见的肌肉骨骼系统疾病。该疾病的特征是骨段骨质溶解,伴有淋巴管局部增生。大量渗漏的全身淋巴管的存在常伴有乳糜性腹水。目前尚无针对戈勒姆病的标准化治疗方法,其分子机制仍不清楚。未来理解戈勒姆病的策略应强调其作为一种淋巴管生成紊乱疾病的明显特征。淋巴研究的突破已经确定了几种可能在戈勒姆病中起相关作用的淋巴管生成途径。

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