{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T06:57:33Z","timestamp":1770793053880,"version":"3.50.0"},"reference-count":24,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,6,30]],"date-time":"2020-06-30T00:00:00Z","timestamp":1593475200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000055","name":"National Institute on Deafness and Other Communication Disorders","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000055","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1K23DC014082"],"award-info":[{"award-number":["1K23DC014082"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1R21DC017225"],"award-info":[{"award-number":["1R21DC017225"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate inheritance patterns and define the familial clustering rate of idiopathic subglottic stenosis (iSGS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Retrospective observational study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Setting<\/jats:title><jats:p>International multicenter collaborative of &gt;30 tertiary care centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with a clinically confirmed iSGS diagnosis within the North American Airway Collaborative\u2019s iSGS<jats:sup>1000<\/jats:sup> cohort consented between 2014 and 2018 were eligible for enrollment. Patient demographics and disease severity were abstracted from the collaborative\u2019s iSGS longitudinal registry. Pedigrees of affected families were created.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 810 patients with iSGS were identified. Positive family history for iSGS was reported in 44 patients in 20 families. The rate of familial clustering in iSGS is 2.5%. Mean age of disease onset is 42.6 years. Of the 44 patients with familial aggregation of iSGS, 42 were female and 2 were male; 13 were mother\u2010daughter pairs and 2 were father\u2010daughter pairs. There were 3 sister\u2010sister pairs. There was 1\u00a0niece\u2010aunt pair and 2\u00a0groups of 3 family members. One pedigree demonstrated 2 affected mother\u2010daughter pairs, with the mothers being first\u2010degree paternal cousins. Inheritance is non\u2010Mendelian, and anticipation is present in 11 of 13 (84%) parent\u2010offspring pairs. The mean age of onset between parents (48.4 years) and offspring (36.1 years) was significantly different (<jats:italic>P<\/jats:italic> =. 016).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study quantifies the rate of familial clustering of iSGS at 2.5%. Inheritance is non\u2010Mendelian, and disease demonstrates anticipation. These data suggest that there may be a genetic contribution in iSGS.<\/jats:p><\/jats:sec>","DOI":"10.1177\/0194599820935402","type":"journal-article","created":{"date-parts":[[2020,6,30]],"date-time":"2020-06-30T04:21:31Z","timestamp":1593490891000},"page":"1011-1017","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Familial Aggregation in Idiopathic Subglottic Stenosis"],"prefix":"10.1002","volume":"163","author":[{"given":"Virginia E.","family":"Drake","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Johns Hopkins University Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Gelbard","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Vanderbilt University Medical Center Nashville Tennessee 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