Gorham病的发病机制  

Pathogenesis of Gorham＇s disease

作　　者：何晓东[1] 夏维波[1] 姜艳[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院内分泌科卫生部内分泌重点实验室,北京100730

出　　处：《国际内分泌代谢杂志》2009年第B04期69-72,共4页International Journal of Endocrinology and Metabolism

摘　　要：Gorham病（即大块骨溶解症）是一种少见、病因不明的疾病，以骨溶解为突出表现，并因此可产生一系列并发症及临床后果。病理改变主要包括骨溶解（或被替代）及进行性、非肿瘤性的毛细血管和（或）毛细淋巴管增殖两个方面。关于前者，大多数研究者认为系破骨细胞数目增加和（或）活性增强所致；关于后者，血小板源性生长因子受体（PDGFR）信号通路机制较令人信服。以上两方面可能存在目前尚未明了的某种内在联系机制。Gorham＇s disease （massive osteolysis） is a rare disease of uncertain etiology. Its predom- inant manifestation is osteolysis which can cause a diverse of complications and clinical outcomes. The patho-logical features involved in Gotham＇s disease include mainly two aspects which may relate with each other, respectively the osteolysis（ or the replacement of normal bone） and the aggressively expanding but non-neoplastic vascular or lymphatic vessel. The activation of osteoclasts is supposed to induce the former in majority＇s opinion, while concerning the etiology of the latter, the hypothesis of platelet-derived growth factor receptor signal pathway seems to be relatively credible.

关 键 词：GORHAM病 发病机制 血小板源性生长因子受体 大块骨溶解症 

分 类 号：R782[医药卫生—口腔医学] R774.1[医药卫生—临床医学]