{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T16:59:30Z","timestamp":1781542770937,"version":"3.54.5"},"reference-count":0,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2019,12,16]],"date-time":"2019-12-16T00:00:00Z","timestamp":1576454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Advances in Respiratory Medicine"],"abstract":"<jats:p>Birt-Hogg-Dub\u00e9 syndrome (BHDS) is an uncommon autosomal dominant syndrome. It is also known as Hornstein\u2013Knickenberg syndrome. It is an inherited disorder culminating in mutations in folliculin coding gene (FLCN). The clinical exhibitions of the syn-drome are multi-systemic, comprising of a constellation of pulmonary, dermatologic and renal system manifestations. The most common presentations include fibrofolliculomas, renal cell carcinomas, lung cysts and spontaneous pneumothorax. The treatment is conservative with regular monitoring of the renal and lung parameters. Fibrofolliculomas may require surgical excision and recurrent events of pneumothorax may warrant pleurodesis. We reported a case series of 2 patients presenting with symptoms of progressive breathlessness along with dermatological manifestations and subsequently showing radiological manifestations of Birt-Hogg-Dub\u00e9 syndrome in the form of lung cysts.<\/jats:p>","DOI":"10.5603\/arm.a2020.0180","type":"journal-article","created":{"date-parts":[[2021,1,20]],"date-time":"2021-01-20T14:41:08Z","timestamp":1611153668000},"page":"55-59","source":"Crossref","is-referenced-by-count":2,"title":["Birt-Hogg-Dub\u00e9 Syndrome\u2014An Unique Case Series"],"prefix":"10.3390","volume":"89","author":[{"given":"Parikshit","family":"Thakare","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ketaki","family":"Utpat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Unnati","family":"Desai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chitra","family":"Nayak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jyotsna M.","family":"Joshi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,12,16]]},"container-title":["Advances in Respiratory Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2543-6031\/89\/1\/55\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T09:35:11Z","timestamp":1657272911000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2543-6031\/89\/1\/55"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12,16]]},"references-count":0,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,2]]}},"alternative-id":["ARM.a2020.0180"],"URL":"https:\/\/doi.org\/10.5603\/arm.a2020.0180","relation":{},"ISSN":["2543-6031"],"issn-type":[{"value":"2543-6031","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,12,16]]}}}