See the published version of this preprint at Orphanet Journal of Rare Diseases.
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Xinlun Tian
Corresponding Author
ORCiD: https://orcid.org/0000-0003-4307-6665
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Background and objective: The purpose of this study was to create a practical CT-based algorithm to differentiate Birt-Hogg-Dubé (BHD) syndrome from other diffuse cystic lung diseases (DCLD).
Methods: The study was a retrospective review of the CT images of 33 patients with BHD syndrome, 33 patients with LAM, and 23 patients with NBNL (non-BHD and non-LAM) among DCLD patients. On the basis of the data collected, the CT images were reviewed again to evaluate the characteristics (size, number, distribution, and morphology) of pulmonary cysts.
Results: Lower lung-predominant cysts were more likely to be found in patients with BHD syndrome than in patients with LAM or in the NBNL DCLD group. In the axial distribution, 18 of 33 patients in BHD group had cysts that were predominantly near the mediastinum, and all the patients in the LAM and NBNL DCLD groups had diffuse cysts. The appearance of fusiform cysts was more easily observed in patients in the BHD group. In total, 58% patients in the BHD group had less than 50 lung cysts, while all patients in the non-BHD group had more than 50 lung cysts. The biggest cyst was located in the lower lobe in 28 of 33 patients in the BHD group, while 11 of 33 patients in LAM group and 10 patients in the NBNL DCLD group had the biggest cyst in the lower lobe.
Conclusion: The pulmonary cysts in patients with BHD tended to be fusiform, less numerous and located predominantly in the lower lobe and near the mediastinum. These radiologic pulmonary features could assist physicians in differentiating BHD from other DCLDs.
Keywords
Birt-Hogg-Dubé syndrome, FLCN, image, diffuse cystic lung diseases, radiology
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CURRENT STATUS: Published
View the published article at Orphanet Journal of Rare Diseases.
Version 2
Posted 09 Jun, 2020
No community comments so far
Editorial decision: Accept
On 21 Jun, 2020
Review #1 received
Received 09 Jun, 2020
Editor assigned
On 03 Jun, 2020
Reviewers invited
Invitations sent on 03 Jun, 2020
Reviewer #1 agreed
On 03 Jun, 2020
Submission checks complete
On 02 Jun, 2020
Editor invited
On 02 Jun, 2020
No comments provided
Editorial decision: Major revision
On 10 May, 2020
Review #2 received
Received 08 May, 2020
Reviewer #2 agreed
On 21 Apr, 2020
Review #1 received
Received 13 Feb, 2020
Reviewer #1 agreed
On 27 Jan, 2020
Reviewers invited
Invitations sent on 02 Jan, 2020
Editor assigned
On 23 Dec, 2019
First submitted
On 22 Dec, 2019
Submission checks complete
On 22 Dec, 2019
Editor invited
On 22 Dec, 2019
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Internal Medicine
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See the published version of this preprint at Orphanet Journal of Rare Diseases.
This is a preprint, a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research
Xinlun Tian
Corresponding Author
ORCiD: https://orcid.org/0000-0003-4307-6665
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Published
View the published article at Orphanet Journal of Rare Diseases.
Version 2
Posted 09 Jun, 2020
No community comments so far
Editorial decision: Accept
On 21 Jun, 2020
Review #1 received
Received 09 Jun, 2020
Editor assigned
On 03 Jun, 2020
Reviewers invited
Invitations sent on 03 Jun, 2020
Reviewer #1 agreed
On 03 Jun, 2020
Submission checks complete
On 02 Jun, 2020
Editor invited
On 02 Jun, 2020
No comments provided
Editorial decision: Major revision
On 10 May, 2020
Review #2 received
Received 08 May, 2020
Reviewer #2 agreed
On 21 Apr, 2020
Review #1 received
Received 13 Feb, 2020
Reviewer #1 agreed
On 27 Jan, 2020
Reviewers invited
Invitations sent on 02 Jan, 2020
Editor assigned
On 23 Dec, 2019
First submitted
On 22 Dec, 2019
Submission checks complete
On 22 Dec, 2019
Editor invited
On 22 Dec, 2019
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Internal Medicine
License:
This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at Orphanet Journal of Rare Diseases.
Background and objective: The purpose of this study was to create a practical CT-based algorithm to differentiate Birt-Hogg-Dubé (BHD) syndrome from other diffuse cystic lung diseases (DCLD).
Methods: The study was a retrospective review of the CT images of 33 patients with BHD syndrome, 33 patients with LAM, and 23 patients with NBNL (non-BHD and non-LAM) among DCLD patients. On the basis of the data collected, the CT images were reviewed again to evaluate the characteristics (size, number, distribution, and morphology) of pulmonary cysts.
Results: Lower lung-predominant cysts were more likely to be found in patients with BHD syndrome than in patients with LAM or in the NBNL DCLD group. In the axial distribution, 18 of 33 patients in BHD group had cysts that were predominantly near the mediastinum, and all the patients in the LAM and NBNL DCLD groups had diffuse cysts. The appearance of fusiform cysts was more easily observed in patients in the BHD group. In total, 58% patients in the BHD group had less than 50 lung cysts, while all patients in the non-BHD group had more than 50 lung cysts. The biggest cyst was located in the lower lobe in 28 of 33 patients in the BHD group, while 11 of 33 patients in LAM group and 10 patients in the NBNL DCLD group had the biggest cyst in the lower lobe.
Conclusion: The pulmonary cysts in patients with BHD tended to be fusiform, less numerous and located predominantly in the lower lobe and near the mediastinum. These radiologic pulmonary features could assist physicians in differentiating BHD from other DCLDs.
Figure 1
Figure 2
Figure 3
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