The molecular mechanism of Gorham syndrome: an update

作者全名："Xiang, Juqin; Zhong, Weiyang"

作者地址："[Xiang, Juqin] Chongqing Med Univ, Chongqing, Peoples R China; [Zhong, Weiyang] Chongqing Med Univ, Dept Orthopaed, Affiliated Hosp 1, Chongqing, Peoples R China"

通信作者："Zhong, WY (通讯作者)，Chongqing Med Univ, Dept Orthopaed, Affiliated Hosp 1, Chongqing, Peoples R China."

来源：FRONTIERS IN IMMUNOLOGY

ESI学科分类：IMMUNOLOGY

WOS号：WOS:000990541500001

JCR分区：Q1

影响因子：5.7

年份：2023

卷号：14

期号：　

开始页：　

结束页：　

文献类型：Review

关键词：Gorham syndrome; molecular mechanism; lymphoid proliferation; vascular proliferation; osteolysis treatment

摘要："Gorham syndrome, also known as ""vanishing osteopathy"" and ""invasive hemangiomatosis,"" is a rare clinical syndrome whose etiology is unknown and can invade the whole-body skeleton. At present, more than 300 cases have been reported at home and abroad, usually manifesting as spontaneous chronic osteolysis with no periosteal reaction at the lysis site and occult onset, often with fractures, scoliosis, chylothorax, etc. When waiting for medical treatment, the condition is serious, and the prognosis is poor. At present, there is no effective treatment. The main pathological manifestations of Gorham syndrome are the non-neoplastic abnormal proliferation of lymphatic vessels or blood vessels and osteolysis caused by osteoclast proliferation or increased activity. At present, there is no unified conclusion regarding Gorham syndrome's pathogenesis. This paper starts with the two most studied osteolysis methods at present, osteoclast osteolysis and osteolysis caused by vascular and lymphatic proliferation and summarizes the corresponding most possible molecular mechanisms in recent years to provide more ideas for Gorham syndrome treatment."

基金机构：Chongqing Nature Science Foundation Project [CSTB2022NSCQ-MSX0070]; Chongqing Medical Scientific Research Project (Joint Project of Chongqing Health~Commission Science and Technology Bureau); program for Youth Innovation in Future of Chongqing Medical University [2021MSXM285]; Hospital Training Funding; Chongqing Medical Scientific Research Project (Science and Technology Bureau); [W0012]; [PYJJ2019-08]

基金资助正文："This study was supported by the Chongqing Nature Science Foundation Project (CSTB2022NSCQ-MSX0070), the Hospital Training Funding (PYJJ2019-08), the Chongqing Medical Scientific Research Project (Joint Project of Chongqing Health Commission and Science and Technology Bureau) (2021MSXM285), and the program for Youth Innovation in Future of Chongqing Medical University (W0012). Funding bodies were not involved in the design, data collection, analysis, interpretation, or writing of the manuscript."