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Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report

SUMMARY: Gorham–Stout disease (GSD) is a rare bone disease characterized by massive osteolysis and lymphatic proliferation. The origin of the condition is unknown, and no established treatment protocol exists. Massive pleural effusion is a frequent complication of GSD in the thoracic region. We pres...

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Autores principales: Toivonen, Leevi A, Neva, Marko H, Sioris, Thanos, Isomäki, Pia, Metso, Saara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9175580/
https://www.ncbi.nlm.nih.gov/pubmed/35642669
http://dx.doi.org/10.1530/EDM-21-0101
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author Toivonen, Leevi A
Neva, Marko H
Sioris, Thanos
Isomäki, Pia
Metso, Saara
author_facet Toivonen, Leevi A
Neva, Marko H
Sioris, Thanos
Isomäki, Pia
Metso, Saara
author_sort Toivonen, Leevi A
collection PubMed
description SUMMARY: Gorham–Stout disease (GSD) is a rare bone disease characterized by massive osteolysis and lymphatic proliferation. The origin of the condition is unknown, and no established treatment protocol exists. Massive pleural effusion is a frequent complication of GSD in the thoracic region. We present the case of a 23-year-old male with thoracic GSD, subsequent paraparesis, and life-threatening pleural effusion. The patient was managed by a multidisciplinary team with a good recovery. The pleural effusion was successfully treated with a pleuro-peritoneal shunt. This is the first report of the use of this mini-invasive technique in the management of pleural effusion related to GSD. Further, we present the potential role of interleukin-6 and bone resorption markers in the measurement of the disease activity. LEARNING POINTS: Multidisciplinary approach is important in the management of rare and severe disorders such as Gorham-Stout disease. Pleuro-peritoneal shunting is a valuable option in the treatment of pleural effusion related to GSD. Interleukin-6 and bone resorption markers appear useful in measuring the disease activity of GSD.
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spelling pubmed-91755802022-06-14 Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report Toivonen, Leevi A Neva, Marko H Sioris, Thanos Isomäki, Pia Metso, Saara Endocrinol Diabetes Metab Case Rep Novel Treatment SUMMARY: Gorham–Stout disease (GSD) is a rare bone disease characterized by massive osteolysis and lymphatic proliferation. The origin of the condition is unknown, and no established treatment protocol exists. Massive pleural effusion is a frequent complication of GSD in the thoracic region. We present the case of a 23-year-old male with thoracic GSD, subsequent paraparesis, and life-threatening pleural effusion. The patient was managed by a multidisciplinary team with a good recovery. The pleural effusion was successfully treated with a pleuro-peritoneal shunt. This is the first report of the use of this mini-invasive technique in the management of pleural effusion related to GSD. Further, we present the potential role of interleukin-6 and bone resorption markers in the measurement of the disease activity. LEARNING POINTS: Multidisciplinary approach is important in the management of rare and severe disorders such as Gorham-Stout disease. Pleuro-peritoneal shunting is a valuable option in the treatment of pleural effusion related to GSD. Interleukin-6 and bone resorption markers appear useful in measuring the disease activity of GSD. Bioscientifica Ltd 2022-05-03 /pmc/articles/PMC9175580/ /pubmed/35642669 http://dx.doi.org/10.1530/EDM-21-0101 Text en © The authors https://creativecommons.org/licenses/by-nc-nd/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. (https://creativecommons.org/licenses/by-nc-nd/4.0/) .
spellingShingle Novel Treatment
Toivonen, Leevi A
Neva, Marko H
Sioris, Thanos
Isomäki, Pia
Metso, Saara
Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title_full Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title_fullStr Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title_full_unstemmed Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title_short Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
title_sort gorham–stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report
topic Novel Treatment
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9175580/
https://www.ncbi.nlm.nih.gov/pubmed/35642669
http://dx.doi.org/10.1530/EDM-21-0101
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