{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T02:07:17Z","timestamp":1785809237482,"version":"3.56.0"},"reference-count":20,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2017,11,24]],"date-time":"2017-11-24T00:00:00Z","timestamp":1511481600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health","award":["5UL1TR001067-02"],"award-info":[{"award-number":["5UL1TR001067-02"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:sec><jats:title>Objectives\/Hypothesis<\/jats:title><jats:p>Our objective was to describe epistaxis onset and severity in pediatric hereditary hemorrhagic telangiectasia (HHT) patients and study the cumulative incidence of epistaxis by age of onset within each genetic subtype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Retrospective cohort chart review.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Charts were reviewed of patients age 0 to 18 years with a clinical or genetic diagnosis of HHT who were evaluated at a tertiary multidisciplinary HHT clinic from January 2010 to June 2016. The epistaxis severity score (ESS), a validated tool for assessing epistaxis severity, was used to assess epistaxis. Statistical analyses were conducted on the full HHT cohort as well as subgroups stratified by the HHT causative gene (HHT1 = <jats:italic>ENG<\/jats:italic> and HHT2 = <jats:italic>ACVRL1<\/jats:italic>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010nine pediatric subjects were identified; 60 had HHT confirmed by genetic testing, and nine (from families with known mutations) met published clinical diagnostic criteria alone. Fifty\u2010nine (85%) had onset of epistaxis. The median age of onset of epistaxis was 5 years (interquartile range [IQR]: 2\u20139 years). The median ESS for the entire cohort was 1.6 (IQR: 0\u20132.6). The median ESS was higher in HHT1 versus HHT2 (2.3 vs. 1.1, <jats:italic>P<\/jats:italic> = .002), and age of epistaxis onset was earlier in HHT1 (3 vs. 5 years, <jats:italic>P<\/jats:italic> = .03). Sex and age were not associated with ESS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Epistaxis may present early in HHT, but is typically mild in the pediatric period. Severity in the pediatric population is worse in patients with HHT1. By recognizing the significance of even mild, infrequent epistaxis in a child with a family history of HHT, and understanding that not all HHT patients have epistaxis during childhood, community providers and otolaryngologist can assist in the early detection of HHT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 128:1714\u20131719, 2018<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.27015","type":"journal-article","created":{"date-parts":[[2017,11,24]],"date-time":"2017-11-24T12:44:44Z","timestamp":1511527484000},"page":"1714-1719","source":"Crossref","is-referenced-by-count":21,"title":["Epistaxis in children and adolescents with hereditary hemorrhagic telangiectasia"],"prefix":"10.1002","volume":"128","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6851-6079","authenticated-orcid":false,"given":"Cristian D.","family":"Gonzalez","sequence":"first","affiliation":[{"name":"Department of Internal Medicine University of Utah Affiliated Hospitals Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie","family":"Mcdonald","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Utah Salt Lake City Utah U.S.A"},{"name":"Department of Radiology University of Utah Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Stevenson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics Stanford University Stanford California U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin J.","family":"Whitehead","sequence":"additional","affiliation":[{"name":"George E. Wahlen VA Medical Center Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew G.","family":"Petersen","sequence":"additional","affiliation":[{"name":"University of Utah School of Medicine Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela P.","family":"Presson","sequence":"additional","affiliation":[{"name":"Division of Epidemiology Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Ding","sequence":"additional","affiliation":[{"name":"Division of Epidemiology 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 and Neck Surgery University of Utah Salt Lake City Utah U.S.A"},{"name":"Division of Cardiovascular Medicine, Pediatric Cardiology, Molecular Medicine Program University of Utah Salt Lake City Utah U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,11,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.2307\/1533331"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10026"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2796.1999.00398.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3182136d32"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.02.009"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320320302"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2010-2366"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-005-0956-8"},{"key":"e_1_2_6_10_1","first-page":"163","article-title":"Hereditary hemorrhagic telangiectasia: children need screening too","volume":"37","author":"Sekarski LA","year":"2011","journal-title":"Pediatr Nurs"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-199109000-00008"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31101"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.40.8.585"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.25604"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.109.1.e12"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.069013"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e31821d2e6d"},{"key":"e_1_2_6_18_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_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20818"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2011.25.3616"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.2174\/138161206776361192"}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.27015","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.27015","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T13:34:51Z","timestamp":1694957691000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.27015"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,11,24]]},"references-count":20,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2018,7]]}},"alternative-id":["10.1002\/lary.27015"],"URL":"https:\/\/doi.org\/10.1002\/lary.27015","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,11,24]]}}}