{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T00:57:56Z","timestamp":1782521876803,"version":"3.54.5"},"reference-count":12,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,9,15]],"date-time":"2015-09-15T00:00:00Z","timestamp":1442275200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01NS075168"],"award-info":[{"award-number":["R01NS075168"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"name":"National Institutes of Health\/National Heart, Lung, and Blood Institute","award":["T35 HL007744, PI: Jerry Kaplan"],"award-info":[{"award-number":["T35 HL007744, PI: Jerry Kaplan"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2016,4]]},"abstract":"<jats:sec><jats:title>Objectives\/Hypothesis<\/jats:title><jats:p>Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia whose hallmark symptom is spontaneous recurrent epistaxis. Two major genetic subtypes of this syndrome are HHT1 and HHT2. Severity of epistaxis ranges from occasional low\u2010volume bleeding to frequent large\u2010volume hemorrhage. This study evaluated the severity and progression of epistaxis in HHT1 versus HHT2.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Retrospective cohort study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective chart review was performed for 183 genotyped HHT patients seen at our center from 2010 to 2013. Data collected included epistaxis severity score (ESS), age of epistaxis onset, number and type of treatments, age at which treatments were sought, complete blood count values, ferritin, number of telangiectases, blood transfusions, iron therapy history, and patient demographics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>115 subjects with HHT2 were compared to 68 with HHT1. Subjects with HHT2 had a higher ESS compared to HHT1 (<jats:italic>P<\/jats:italic>\u2009=\u2009.043) and a later age of onset of epistaxis (<jats:italic>P<\/jats:italic>\u2009=\u2009.005). HHT2 subjects were more likely to use oral iron (<jats:italic>P<\/jats:italic>\u2009=\u2009.032) and were more likely to seek interventions to control their epistaxis (<jats:italic>P<\/jats:italic>\u2009=\u2009.029).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>HHT2 is associated with more severe epistaxis and a subsequent higher rate of interventions, requiring more aggressive therapy as compared to HHT1.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 126:786\u2013790, 2016<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.25604","type":"journal-article","created":{"date-parts":[[2015,9,15]],"date-time":"2015-09-15T13:15:01Z","timestamp":1442322901000},"page":"786-790","source":"Crossref","is-referenced-by-count":12,"title":["An evaluation of the severity and progression of epistaxis in hereditary hemorrhagic telangiectasia 1 versus hereditary hemorrhagic telangiectasia 2"],"prefix":"10.1002","volume":"126","author":[{"given":"Benjamin N.","family":"Hunter","sequence":"first","affiliation":[{"name":"University of Utah School of Medicine Salt Lake City Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin H.","family":"Timmins","sequence":"additional","affiliation":[{"name":"University of Utah School of Medicine Salt Lake City Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie","family":"McDonald","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Utah Salt Lake City Utah"},{"name":"Department of Radiology University of Utah Salt Lake City Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin J.","family":"Whitehead","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine, Pediatric Cardiology, Molecular Medicine Program University of Utah Salt Lake City Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. Daniel","family":"Ward","sequence":"additional","affiliation":[{"name":"Division of Otolaryngology\u2013Head &amp; Neck Surgery University of Utah Salt Lake City Utah U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin F.","family":"Wilson","sequence":"additional","affiliation":[{"name":"Division of Otolaryngology\u2013Head &amp; Neck Surgery University of Utah Salt Lake City Utah U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,9,15]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.069013"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0696-189"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1294-345"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.GIM.0000132689.25644.7C"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.40.8.585"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-199109000-00008"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32243"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e31802d8373"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31101"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20818"},{"key":"e_1_2_6_13_1","unstructured":"GossageJ;Hereditary Hemorrhagic Telangiectasia Foundation International. North American Study of epistaxis in HHT. ClinicalTrials.gov website. Identifier: NCT01408030. Available at:https:\/\/clinicaltrials.gov\/ct2\/show\/NCT01408030NLM accessed July 11 2015."}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.25604","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.25604","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.25604","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T01:40:29Z","timestamp":1696556429000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.25604"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,9,15]]},"references-count":12,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2016,4]]}},"alternative-id":["10.1002\/lary.25604"],"URL":"https:\/\/doi.org\/10.1002\/lary.25604","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,9,15]]}}}