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

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, strati

SUBMITTER: Muller ME 

PROVIDER: S-EPMC8111214 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature