A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome

Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pne...

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Detalles Bibliográficos
Autores principales: Minnis, P., Riddell, P., Keane, M.P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4900472/
https://www.ncbi.nlm.nih.gov/pubmed/27330962
http://dx.doi.org/10.1016/j.rmcr.2016.05.007
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author Minnis, P.
Riddell, P.
Keane, M.P.
author_facet Minnis, P.
Riddell, P.
Keane, M.P.
author_sort Minnis, P.
collection PubMed
description Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pneumothorax of 50 times greater than the normal population. We describe here a case of Birt-Hogg-Dubé diagnosed in the 9th decade of life and discuss the radiological findings and clinical implications.
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spelling pubmed-49004722016-06-21 A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome Minnis, P. Riddell, P. Keane, M.P. Respir Med Case Rep Case Report Birt-Hogg-Dubé syndrome, initially described in 1977, is an autosomal dominant inherited condition characterised by basal pulmonary cysts often resulting in pneumothorax, renal tumours and cutaneous involvement. Lung cysts have been described in up to 90% of patients with a corresponding risk of pneumothorax of 50 times greater than the normal population. We describe here a case of Birt-Hogg-Dubé diagnosed in the 9th decade of life and discuss the radiological findings and clinical implications. Elsevier 2016-05-29 /pmc/articles/PMC4900472/ /pubmed/27330962 http://dx.doi.org/10.1016/j.rmcr.2016.05.007 Text en © 2016 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Minnis, P.
Riddell, P.
Keane, M.P.
A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title_full A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title_fullStr A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title_full_unstemmed A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title_short A rare cause of cystic lung disease – Birt-Hogg-Dubé syndrome
title_sort rare cause of cystic lung disease – birt-hogg-dubé syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4900472/
https://www.ncbi.nlm.nih.gov/pubmed/27330962
http://dx.doi.org/10.1016/j.rmcr.2016.05.007
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