WebBirt-Hogg-Dubé (BHD) syndrome is an uncommon, autosomal dominant, multiorgan systemic disorder manifesting as cutaneous fibrofolliculomas, lung cysts with or without spontaneous pneumothorax, and renal tumors. Spontaneous pneumothorax and lung cysts on chest computed tomography (CT) should lead to t … Birt-Hogg-Dubé syndrome is rare but likely under-diagnosed. It may be diagnosed incidentally after a CT scan, dermatological review or a spontaneous pneumothorax. Men and women are equally affected as this an autosomal dominant disorder. There is often a family history of pneumothorax. See more One major criterion: 1. five adult-onset fibrofolliculomas 2. pathogenic FLCNgermline mutation Two minor criteria: 1. typical lung cysts with no other explanation 2. multifocal/bilateral renal cancer before the … See more Lung cysts typically develop in early adulthood and have the following characteristics: 1. multiple lower zone predominant and bilateral 2. predilection for subpleural lung including paramediastinal and … See more Folliculin is thought to be an oncogene suppressor protein which may affect proteolytic metalloproteinase enzymes leading to lung matrix … See more

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WebJul 6, 2024 · 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 … WebBirt-Hogg-Dubé syndrome is a rare autosomal-dominant disorder characterized by fibrofolliculomas distributed over the face, neck, and upper trunk; renal tumors ranging from benign oncocytomas to renal cell carcinomas; colonic … fly from newcastle to cardiff

Characterization of CT scans of patients with Birt-Hogg-Dubé

WebIn our initial search, 155 studies containing the terms “Birt-Hogg-Dubé” or “BHD” in the radiology report were identified ... Park HJ, Park CH, Lee SE, et al. Birt-Hogg-Dube syndrome prospectively detected by review of chest computed tomography scans. PLoS One 2024; 12:e0170713 [Google Scholar] WebDiscrete thin-walled cysts in patients with Birt-Hogg-Dubé syndrome are more numerous and larger in the lower lobes and vary in size and shape. Large lung cysts are frequently multiseptated. These features may aid in differentiating Birt-Hogg-Dubé syndrome from other more common cystic lung diseases. WebRadiologists should be aware of the association between these rare disorders. 1. Introduction Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disease characterized by skin fibrofolliculomas, pulmonary cysts, spontaneous pneumothoraces, and renal cancers [ 1 ]. green leaf frenchies scam