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Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases
Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone. Typical bone-related symptoms include swelling, pain and functional impairment in the region involved. The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblas...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Bioscientifica Ltd
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8788153/ https://www.ncbi.nlm.nih.gov/pubmed/35076412 http://dx.doi.org/10.1530/EOR-21-0083 |
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author | Angelini, Andrea Mosele, Nicolò Pagliarini, Elisa Ruggieri, Pietro |
author_facet | Angelini, Andrea Mosele, Nicolò Pagliarini, Elisa Ruggieri, Pietro |
author_sort | Angelini, Andrea |
collection | PubMed |
description | Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone. Typical bone-related symptoms include swelling, pain and functional impairment in the region involved. The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblast function. Multi-targeted pharmacological approach includes innovative options and represent milestones of treatment, sometimes associated with radiotherapy. Surgery is mainly used to treat complications: pathologic/impending fractures, spinal instability or deformities and chylothorax. In this narrative review, we highlight current standards in diagnosis, clinical management and therapeutic strategies. |
format | Online Article Text |
id | pubmed-8788153 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Bioscientifica Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-87881532022-01-28 Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases Angelini, Andrea Mosele, Nicolò Pagliarini, Elisa Ruggieri, Pietro EFORT Open Rev General Orthopaedics Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone. Typical bone-related symptoms include swelling, pain and functional impairment in the region involved. The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblast function. Multi-targeted pharmacological approach includes innovative options and represent milestones of treatment, sometimes associated with radiotherapy. Surgery is mainly used to treat complications: pathologic/impending fractures, spinal instability or deformities and chylothorax. In this narrative review, we highlight current standards in diagnosis, clinical management and therapeutic strategies. Bioscientifica Ltd 2022-01-11 /pmc/articles/PMC8788153/ /pubmed/35076412 http://dx.doi.org/10.1530/EOR-21-0083 Text en © The authors https://creativecommons.org/licenses/by-nc/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. (https://creativecommons.org/licenses/by-nc/4.0/) |
spellingShingle | General Orthopaedics Angelini, Andrea Mosele, Nicolò Pagliarini, Elisa Ruggieri, Pietro Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title | Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title_full | Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title_fullStr | Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title_full_unstemmed | Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title_short | Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases |
title_sort | current concepts from diagnosis to management in gorham–stout disease: a systematic narrative review of about 350 cases |
topic | General Orthopaedics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8788153/ https://www.ncbi.nlm.nih.gov/pubmed/35076412 http://dx.doi.org/10.1530/EOR-21-0083 |
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