The pathogenesis of pneumothorax in Birt-Hogg-Dub, syndrome: A hypothesis

被引：20
|
作者：
Johannesma, Paul Christiaan [1 ]
Houweling, Arjan C. [2 ]
van Waesberghe, Jan-Hein T. M. [3 ]
van Moorselaar, R. J. Jeroen A. [4 ]
Starink, Theo M. [5 ]
Menko, Fred H. [2 ,6 ]
Postmus, Pieter E. [1 ]
机构：
[1] Vrije Univ Amsterdam Med Ctr, Dept Pulm Dis, NL-1007 MB Amsterdam, Netherlands
[2] Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, NL-1007 MB Amsterdam, Netherlands
[3] Vrije Univ Amsterdam Med Ctr, Dept Radiol, NL-1007 MB Amsterdam, Netherlands
[4] Vrije Univ Amsterdam Med Ctr, Dept Urol, NL-1007 MB Amsterdam, Netherlands
[5] Vrije Univ Amsterdam Med Ctr, Dept Dermatol, NL-1007 MB Amsterdam, Netherlands
[6] Netherlands Canc Inst, Family Canc Clin, Amsterdam, Netherlands
来源：
RESPIROLOGY | 2014年 / 19卷 / 08期
关键词：
Birt-Hogg-Dube syndrome; folliculin; folliculin gene; pneumothorax; renal cell cancer; CYSTIC LUNG-DISEASE; PULMONARY CYSTS; GENE;
D O I：
10.1111/resp.12405
中图分类号：
R56 [呼吸系及胸部疾病];
学科分类号：
摘要：
The development and natural course of lung cysts in patients with Birt-Hogg-Dube syndrome (BHD) is still unclear, and the relationship between lung cysts and pneumothorax is not fully clarified. Based on the follow-up results of thoracic imaging in six patients with BHD, we hypothesize that decreased potential for stretching of the cysts' wall and extensive contact with the visceral pleura are probably responsible for rupture of the cyst wall resulting in increased risk for pneumothorax.
引用
收藏
页码：1248 / 1250
页数：3
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