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Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients

Abstract

Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome. Materials and methods: Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the {kappa} statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient. Results: Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients. Conclusion: Multiple, irregular-shaped cysts of various sizes with lower medial lung  More>>
Authors:
Tobino, Kazunori; [1]  Gunji, Yoko; [1]  Kurihara, Masatoshi; [2]  The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]; Kunogi, Makiko; Koike, Kengo; [1]  Tomiyama, Noriyuki; [3]  Johkoh, Takeshi; [4]  Kodama, Yuzo; [1]  Iwakami, Shin-ichiro; [5]  Kikkawa, Mika; [6]  Takahashi, Kazuhisa; [1]  Seyama, Kuniaki; [1]  Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]
  1. Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan)
  2. Pneumothorax Center, Nissan Tamagawa Hospital, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)
  3. Department of Radiology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871 (Japan)
  4. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Kurumazuka 3-1, Itami, Hyogo 664-0872 (Japan)
  5. Department of Respiratory Medicine, Juntendo University, Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka 410-2295 (Japan)
  6. Biochemical Research Center, Division of Proteomics and Biomolecular Sciences, Juntendo University, Graduate School of Medicine, Bunkyo-Ku, Tokyo (Japan)
Publication Date:
Mar 15, 2011
Product Type:
Journal Article
Resource Relation:
Journal Name: European Journal of Radiology; Journal Volume: 77; Journal Issue: 3; Other Information: DOI: 10.1016/j.ejrad.2009.09.004; PII: S0720-048X(09)00523-3; Copyright (c) 2009 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved.
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; ARTERIES; CAT SCANNING; CHEST; CYSTS; DISTRIBUTION; LUNGS; PATIENTS; PLEURA; VEINS; BLOOD VESSELS; BODY; CARDIOVASCULAR SYSTEM; COMPUTERIZED TOMOGRAPHY; DIAGNOSTIC TECHNIQUES; MEMBRANES; ORGANS; PATHOLOGICAL CHANGES; RESPIRATORY SYSTEM; SEROUS MEMBRANES; TOMOGRAPHY
OSTI ID:
21526198
Country of Origin:
Netherlands
Language:
English
Other Identifying Numbers:
Journal ID: ISSN 0720-048X; EJRADR; TRN: NL11R0943005981
Availability:
Available from http://dx.doi.org/10.1016/j.ejrad.2009.09.004
Submitting Site:
NLN
Size:
page(s) 403-409
Announcement Date:
Feb 23, 2012

Citation Formats

Tobino, Kazunori, Gunji, Yoko, Kurihara, Masatoshi, The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)], Kunogi, Makiko, Koike, Kengo, Tomiyama, Noriyuki, Johkoh, Takeshi, Kodama, Yuzo, Iwakami, Shin-ichiro, Kikkawa, Mika, Takahashi, Kazuhisa, Seyama, Kuniaki, and Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]. Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients. Netherlands: N. p., 2011. Web. doi:10.1016/j.ejrad.2009.09.004.
Tobino, Kazunori, Gunji, Yoko, Kurihara, Masatoshi, The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)], Kunogi, Makiko, Koike, Kengo, Tomiyama, Noriyuki, Johkoh, Takeshi, Kodama, Yuzo, Iwakami, Shin-ichiro, Kikkawa, Mika, Takahashi, Kazuhisa, Seyama, Kuniaki, & Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]. Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients. Netherlands. https://doi.org/10.1016/j.ejrad.2009.09.004
Tobino, Kazunori, Gunji, Yoko, Kurihara, Masatoshi, The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)], Kunogi, Makiko, Koike, Kengo, Tomiyama, Noriyuki, Johkoh, Takeshi, Kodama, Yuzo, Iwakami, Shin-ichiro, Kikkawa, Mika, Takahashi, Kazuhisa, Seyama, Kuniaki, and Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]. 2011. "Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients." Netherlands. https://doi.org/10.1016/j.ejrad.2009.09.004.
@misc{etde_21526198,
title = {Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients}
author = {Tobino, Kazunori, Gunji, Yoko, Kurihara, Masatoshi, The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)], Kunogi, Makiko, Koike, Kengo, Tomiyama, Noriyuki, Johkoh, Takeshi, Kodama, Yuzo, Iwakami, Shin-ichiro, Kikkawa, Mika, Takahashi, Kazuhisa, Seyama, Kuniaki, and Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)]}
abstractNote = {Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome. Materials and methods: Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the {kappa} statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient. Results: Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients. Conclusion: Multiple, irregular-shaped cysts of various sizes with lower medial lung zone predominance are characteristic CT findings of BHD syndrome. Cysts abutting or including the proximal portions of lower pulmonary arteries or veins may also exist in this syndrome in a high probability.}
doi = {10.1016/j.ejrad.2009.09.004}
journal = []
issue = {3}
volume = {77}
place = {Netherlands}
year = {2011}
month = {Mar}
}