Pulmonary Manifestations and Management of Proteus Syndrome
Proteus syndrome is a very rare, sporadic and congenital condition that is characterized by postnatal mosaic overgrowth. This disorder is thought to be caused by a somatic gene mutation, but the exact etiology is unknown. Commonly involved tissues include connective tissue, bone, skin and the centra...
Main Authors: | , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Elsevier
2010-05-01
|
Series: | Journal of the Formosan Medical Association |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0929664610600691 |
id |
doaj-86569f72f92841fabf4c4d668eea7501 |
---|---|
record_format |
Article |
spelling |
doaj-86569f72f92841fabf4c4d668eea75012020-11-24T20:58:01ZengElsevierJournal of the Formosan Medical Association0929-66462010-05-01109539740010.1016/S0929-6646(10)60069-1Pulmonary Manifestations and Management of Proteus SyndromeChia-Ying Li0Yih-Leong Chang1Wei-Chou Chen2Yung-Chie Lee3Division of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, TaiwanDivision of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, TaiwanDivision of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, TaiwanProteus syndrome is a very rare, sporadic and congenital condition that is characterized by postnatal mosaic overgrowth. This disorder is thought to be caused by a somatic gene mutation, but the exact etiology is unknown. Commonly involved tissues include connective tissue, bone, skin and the central nervous system. Another less common symptom involves pulmonary emphysematous changes. This report documents a 25-year-old man with Proteus syndrome who presented with progressive exertional dyspnea and asymmetric overgrowth of his extremities. He underwent left pneumonectomy and his postoperative course was uneventful. Lung tissue showed emphysematous changes with multiple bulla formation and scattered calcification. We also review recent literature related to pulmonary manifestations and management of Proteus syndrome.http://www.sciencedirect.com/science/article/pii/S0929664610600691bullous lesionmutationpneumonectomypneumothoraxProteus syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Chia-Ying Li Yih-Leong Chang Wei-Chou Chen Yung-Chie Lee |
spellingShingle |
Chia-Ying Li Yih-Leong Chang Wei-Chou Chen Yung-Chie Lee Pulmonary Manifestations and Management of Proteus Syndrome Journal of the Formosan Medical Association bullous lesion mutation pneumonectomy pneumothorax Proteus syndrome |
author_facet |
Chia-Ying Li Yih-Leong Chang Wei-Chou Chen Yung-Chie Lee |
author_sort |
Chia-Ying Li |
title |
Pulmonary Manifestations and Management of Proteus Syndrome |
title_short |
Pulmonary Manifestations and Management of Proteus Syndrome |
title_full |
Pulmonary Manifestations and Management of Proteus Syndrome |
title_fullStr |
Pulmonary Manifestations and Management of Proteus Syndrome |
title_full_unstemmed |
Pulmonary Manifestations and Management of Proteus Syndrome |
title_sort |
pulmonary manifestations and management of proteus syndrome |
publisher |
Elsevier |
series |
Journal of the Formosan Medical Association |
issn |
0929-6646 |
publishDate |
2010-05-01 |
description |
Proteus syndrome is a very rare, sporadic and congenital condition that is characterized by postnatal mosaic overgrowth. This disorder is thought to be caused by a somatic gene mutation, but the exact etiology is unknown. Commonly involved tissues include connective tissue, bone, skin and the central nervous system. Another less common symptom involves pulmonary emphysematous changes. This report documents a 25-year-old man with Proteus syndrome who presented with progressive exertional dyspnea and asymmetric overgrowth of his extremities. He underwent left pneumonectomy and his postoperative course was uneventful. Lung tissue showed emphysematous changes with multiple bulla formation and scattered calcification. We also review recent literature related to pulmonary manifestations and management of Proteus syndrome. |
topic |
bullous lesion mutation pneumonectomy pneumothorax Proteus syndrome |
url |
http://www.sciencedirect.com/science/article/pii/S0929664610600691 |
work_keys_str_mv |
AT chiayingli pulmonarymanifestationsandmanagementofproteussyndrome AT yihleongchang pulmonarymanifestationsandmanagementofproteussyndrome AT weichouchen pulmonarymanifestationsandmanagementofproteussyndrome AT yungchielee pulmonarymanifestationsandmanagementofproteussyndrome |
_version_ |
1716786764307234816 |