Document Detail

Ehlers-Danlos syndrome with recurrent spontaneous pneumothoraces and cavitary lesion on chest X-ray as the initial complications.
MedLine Citation:
PMID:  19420820     Owner:  NLM     Status:  MEDLINE    
A 17-year-old-man developed left-sided pneumothorax in 1995. Chest computed tomography (CT) showed a thick-walled cavity in the left lower lobe. Video-assisted thoracic surgery was performed, and pathologic findings of the resected lung showed a cavity, organizing hematoma, and a fibrous nodule. Fragility of connective tissue was suspected, and biochemical and molecular analysis showed reduction of type III collagen production and point mutation of the COL3A1 gene. The patient was diagnosed as having vascular-type Ehlers-Danlos syndrome (EDS). From 2002, the patient developed hemoptysis and bloody sputum once a year. Chest CT detected several nodules and cavities, which were regarded as hematomas with or without excretion. Several vascular changes including aneurysmal formations have been found since 2002, and an aneurysm of the left ulnar artery was resected. The patient continues to be followed regularly on an outpatient basis. We report a rare case of vascular-type EDS who developed pulmonary symptoms as an initial complication.
Takashi Ishiguro; Noboru Takayanagi; Yoshinori Kawabata; Hidekazu Matsushima; Yutaka Yoshii; Keiji Harasawa; Shozaburo Yamaguchi; Koichiro Yoneda; Yousuke Miyahara; Naho Kagiyama; Daido Tokunaga; Fumiaki Aoki; Hiroo Saito; Kazuyoshi Kurashima; Mikio Ubukata; Tsutomu Yanagisawa; Yutaka Sugita; Hiroshi Okita; Atsushi Hatamochi
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-05-01
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  48     ISSN:  1349-7235     ISO Abbreviation:  Intern. Med.     Publication Date:  2009  
Date Detail:
Created Date:  2009-05-07     Completed Date:  2010-02-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  717-22     Citation Subset:  IM    
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Kumagaya.
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MeSH Terms
Ehlers-Danlos Syndrome / complications,  radiography*
Lung Diseases / complications,  radiography*
Pneumothorax / complications,  radiography*

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