Birt-Hogg-Dube′증후군에서 나타난 자발성 기흉: 2 증례Spontaneous Pneumothorax in Birt-Hogg-Dube′ Syndrome: Two Case
- Other Titles
- Spontaneous Pneumothorax in Birt-Hogg-Dube′ Syndrome: Two Case
- Authors
- 배형주; 우옥희; 용환석; 강은영; 김현구; 최영호; 신봉경; 김윤경
- Issue Date
- 2011
- Publisher
- 대한영상의학회
- Keywords
- Lung; Cysts; Pneumothorax
- Citation
- 대한영상의학회지, v.64, no.1, pp.39 - 43
- Indexed
- KCI
- Journal Title
- 대한영상의학회지
- Volume
- 64
- Number
- 1
- Start Page
- 39
- End Page
- 43
- URI
- https://scholar.korea.ac.kr/handle/2021.sw.korea/113491
- ISSN
- 1738-2637
- Abstract
- Birt-Hogg-Dube′(BHD) syndrome is a rare autosomal dominant inherited disorder that is characterized by skin fibrofolliculomas, renal tumors and multiple lung cysts with or without spontaneous pneumothorax. The disease is caused by germline mutations in the FLCN gene that codes for a protein of unknown function called folliculin. Patients with BHD syndrome do not always have all three manifestations of the skin, kidney and lung. To the best of our knowledge, there has been no case report of the radiologic findings of the lung manifestation in a patient with BHD syndrome in Korea. We report here on two cases of BHD syndrome that presented with spontaneous pneumothorax. The pulmonary abnormalities consisted of multiple thin-walled cysts of various sizes and shapes in both lungs.
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Collections - College of Medicine > Department of Medical Science > 1. Journal Articles
- Graduate School > Department of Biomedical Sciences > 1. Journal Articles
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