Novel clinical scoring system to identify patients with pneumothorax with suspicion for Birt–Hogg–Dubé syndrome

Novel clinical scoring system to identify patients with pneumothorax with suspicion for... AbbreviationsAICAkaike information criterionAUCarea under the curveBHDSBirt–Hogg–Dubé syndromeCTcomputed tomographyPSPprimary spontaneous pneumothoraxPTXpneumothoraxROCreceiver operating characteristicVATSvideo‐assisted thoracoscopic surgeryINTRODUCTIONBirt–Hogg–Dubé syndrome (BHDS), a hereditary genodermatosis, was first described in 1975 and 1977. Subsequent studies revealed that the candidate gene for BHDS was located on chromosome 17p11.2. This subsequently named FLCN gene is composed of 14 exons and encodes a protein called folliculin. The indications of this syndrome are recognized as multiple lung cysts with recurrent pneumothorax (PTX), fibrofolliculomas of the skin and renal tumours. Among these clinical features, lung manifestations are the earliest to occur, usually at 20–30 years of age, whereas skin and renal manifestations do not appear until the 30s–50s. In particular, PTX presents with sudden‐onset shortness of breath and/or chest pain; therefore, patients with BHDS are often referred to outpatient or emergency departments, as PTX is the first presentation of the disease. Accordingly, pulmonologists and thoracic surgeons have a complex role if they are to establish the earliest possible diagnosis of BHDS, thereby benefitting patients by initiating periodic screenings for renal neoplasia.Primary spontaneous pneumothorax (PSP), the most common type of PTX, is a spontaneously occurring air leakage into the pleural space. The disease usually occurs in thin, tall males at 10–20 years http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Respirology Wiley

Novel clinical scoring system to identify patients with pneumothorax with suspicion for Birt–Hogg–Dubé syndrome

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Publisher
Wiley
Copyright
© 2018 Asian Pacific Society of Respirology
ISSN
1323-7799
eISSN
1440-1843
D.O.I.
10.1111/resp.13191
Publisher site
See Article on Publisher Site

Abstract

AbbreviationsAICAkaike information criterionAUCarea under the curveBHDSBirt–Hogg–Dubé syndromeCTcomputed tomographyPSPprimary spontaneous pneumothoraxPTXpneumothoraxROCreceiver operating characteristicVATSvideo‐assisted thoracoscopic surgeryINTRODUCTIONBirt–Hogg–Dubé syndrome (BHDS), a hereditary genodermatosis, was first described in 1975 and 1977. Subsequent studies revealed that the candidate gene for BHDS was located on chromosome 17p11.2. This subsequently named FLCN gene is composed of 14 exons and encodes a protein called folliculin. The indications of this syndrome are recognized as multiple lung cysts with recurrent pneumothorax (PTX), fibrofolliculomas of the skin and renal tumours. Among these clinical features, lung manifestations are the earliest to occur, usually at 20–30 years of age, whereas skin and renal manifestations do not appear until the 30s–50s. In particular, PTX presents with sudden‐onset shortness of breath and/or chest pain; therefore, patients with BHDS are often referred to outpatient or emergency departments, as PTX is the first presentation of the disease. Accordingly, pulmonologists and thoracic surgeons have a complex role if they are to establish the earliest possible diagnosis of BHDS, thereby benefitting patients by initiating periodic screenings for renal neoplasia.Primary spontaneous pneumothorax (PSP), the most common type of PTX, is a spontaneously occurring air leakage into the pleural space. The disease usually occurs in thin, tall males at 10–20 years

Journal

RespirologyWiley

Published: Jan 1, 2018

Keywords: ; ; ;

References

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