Cargando…
The molecular mechanism of Gorham syndrome: an update
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 osteol...
Autores principales: | , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10196207/ https://www.ncbi.nlm.nih.gov/pubmed/37215116 http://dx.doi.org/10.3389/fimmu.2023.1165091 |
_version_ | 1785044296241512448 |
---|---|
author | Xiang, Juqin Zhong, Weiyang |
author_facet | Xiang, Juqin Zhong, Weiyang |
author_sort | Xiang, Juqin |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-10196207 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-101962072023-05-20 The molecular mechanism of Gorham syndrome: an update Xiang, Juqin Zhong, Weiyang Front Immunol Immunology 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. Frontiers Media S.A. 2023-05-05 /pmc/articles/PMC10196207/ /pubmed/37215116 http://dx.doi.org/10.3389/fimmu.2023.1165091 Text en Copyright © 2023 Xiang and Zhong https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Xiang, Juqin Zhong, Weiyang The molecular mechanism of Gorham syndrome: an update |
title | The molecular mechanism of Gorham syndrome: an update |
title_full | The molecular mechanism of Gorham syndrome: an update |
title_fullStr | The molecular mechanism of Gorham syndrome: an update |
title_full_unstemmed | The molecular mechanism of Gorham syndrome: an update |
title_short | The molecular mechanism of Gorham syndrome: an update |
title_sort | molecular mechanism of gorham syndrome: an update |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10196207/ https://www.ncbi.nlm.nih.gov/pubmed/37215116 http://dx.doi.org/10.3389/fimmu.2023.1165091 |
work_keys_str_mv | AT xiangjuqin themolecularmechanismofgorhamsyndromeanupdate AT zhongweiyang themolecularmechanismofgorhamsyndromeanupdate AT xiangjuqin molecularmechanismofgorhamsyndromeanupdate AT zhongweiyang molecularmechanismofgorhamsyndromeanupdate |