[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: Masayuki Sakaguchi, Hirohisa Gotou, Takashi Nakahara, Megumi Fuke, Kazunori Nishimura, Hiroshi Kuraishi, Mitsuko Furuya
• Journal: Kyobu geka. The Japanese journal of thoracic surgery (Kyobu Geka) Vol. 67 Issue 5 Pg. 415-8 (May 2014) ISSN: 0021-5252 [Print] Japan
• PMID: 24917291 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• FLCN protein, human
• Proto-Oncogene Proteins
• Tumor Suppressor Proteins

Topics

• Aged
• Aortic Dissection (complications, surgery)
• Aortic Aneurysm, Thoracic (complications, surgery)
• Birt-Hogg-Dube Syndrome (complications, genetics)
• Exons
• Female
• Humans
• Proto-Oncogene Proteins (genetics)
• Tomography, X-Ray Computed
• Tumor Suppressor Proteins (genetics)