학술논문
Refractory recurrent hemopneumothorax associated with vascular-type Ehlers-Danlos syndrome / 繰り返す血気胸で診断に至ったEhlers-Danlos syndromeの一例
Document Type
Journal Article
Author
Source
日本呼吸器外科学会雑誌 / The Journal of the Japanese Association for Chest Surgery. 2022, 36(2):167
Subject
Language
Japanese
ISSN
0919-0945
1881-4158
1881-4158
Abstract
Vascular-type Ehlers-Danlos syndrome (vEDS) is a rare inherited autosomal dominant disorder. Although the majority of individuals with vEDS are identified because of family history, those remaining present with major complications such as vascular dissection or rupture, gastrointestinal perforation, or organ rupture. Only a few vEDS patients develop spontaneous pneumothorax as an initial complication.