Prevalence of Birt-Hogg-Dubé Syndrome Determined Through Epidemiological Data on Spontaneous Pneumothorax and Bayes Theorem.

Details

Serval ID
serval:BIB_55462E16A012
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Prevalence of Birt-Hogg-Dubé Syndrome Determined Through Epidemiological Data on Spontaneous Pneumothorax and Bayes Theorem.
Journal
Frontiers in medicine
Author(s)
Muller M.E., Daccord C., Taffé P., Lazor R.
ISSN
2296-858X (Print)
ISSN-L
2296-858X
Publication state
Published
Issued date
2021
Peer-reviewed
Oui
Volume
8
Pages
631168
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Background: Birt-Hogg-Dubé syndrome (BHD) is a rare inherited disorder characterized by cutaneous fibrofolliculomas, multiple pulmonary cysts, recurrent spontaneous pneumothorax (SP), and renal tumors. More than 40 years after its description, the prevalence of BHD in the general population remains unknown. This study aimed at determining the prevalence of BHD by applying the Bayes theorem of conditional probability to epidemiological data on SP. Methods: We performed a meta-analysis of published data on: (1) the probability of having BHD among patients with apparent primary SP (4 studies), (2) the incidence rate of primary SP in the general population (9 studies), and (3) the probability of experiencing a SP in BHD (16 studies). Results were corrected for SP relapses, stratified by gender and year of study publication (before and after 2000), and computed with the Bayes equation. Results: The probability of having BHD among patients with apparent primary SP was 0.09 (95% confidence interval: 0.07, 0.11) or 9%. It was 0.20 (0.14, 0.27) in women and 0.05 (0.04, 0.07) in men. The incidence rate of primary SP in the general population was 8.69 (6.58, 11.46) per 100,000 person-years (p-y). It was 3.44 (2.36, 4.99) per 100,000 p-y in women and 13.96 (10.72, 18.18) per 100,000 p-y in men, and was about 2 times higher in studies published after 2000 than in those published before 2000. The probability of experiencing at least one SP among patients with BHD was 0.43 (0.31, 0.54) or 43%, without gender difference. By combining these data in the Bayes equation, we found a prevalence of BHD in the general population of 1.86 (1.16, 3.00) per million, with values of 1.86 (1.02, 3.39) per million in men, and 1.88 (0.97, 3.63) per million in women. Conclusion: The prevalence of BHD in the general population is about 2 cases per million, without difference between genders.
Keywords
Bayes theorem, Birt-Hogg-Dube syndrome, epidemiology, gender, meta-analysis, pneumothorax, prevalence
Pubmed
Web of science
Open Access
Yes
Create date
29/05/2021 16:17
Last modification date
05/06/2021 6:33
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