title: [Acute type A aortic dissection with Birt-Hogg-Dubé syndrome; report of a case].
abstract: We describe a 76-year-old woman with cardiac tamponade who was admitted to our hospital. She underwent ascending and partial arch aortic replacement to treat acute type A aortic dissection. However, postoperative respiratory failure developed and a chest X-ray revealed right lung pneumothorax. The lung was finally expanded after difficulties with prolonged tube drainage. Chest computed tomography(CT) showed multiple cystic changes in the bilateral lungs. Her sister and her daughter also had a history of spontaneous pneumothorax. We finally diagnosed Birt-Hogg-Dube syndrome after deoxyribonucleic asid(DNA)sequencing of folliculin( FLCN) gene.
authors.affiliation: Department of Cardiovascular Surgery, Nagano Redcross Hospital, Nagoya, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sakaguchi
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Gotou
authors.forename: Takashi
authors.initials: T
authors.lastname: Nakahara
authors.forename: Megumi
authors.initials: M
authors.lastname: Fuke
authors.forename: Kazunori
authors.initials: K
authors.lastname: Nishimura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kuraishi
authors.forename: Mitsuko
authors.initials: M
authors.lastname: Furuya
mesh_terms: D000368:Aged; D000784:Aortic Dissection / Q000150:complications / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000150:complications / Q000601:surgery*; D058249:Birt-Hogg-Dube Syndrome / Q000150:complications* / Q000235:genetics; D005091:Exons; D005260:Female; D006801:Humans; D011518:Proto-Oncogene Proteins / Q000235:genetics; D014057:Tomography, X-Ray Computed; D025521:Tumor Suppressor Proteins / Q000235:genetics
chemical_list: C476078:FLCN protein, human; D011518:Proto-Oncogene Proteins; D025521:Tumor Suppressor Proteins