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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...

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Autores principales: Xiang, Juqin, Zhong, Weiyang
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
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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.
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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
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