{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:40:12Z","timestamp":1747183212689,"version":"3.40.5"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T00:00:00Z","timestamp":1635724800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ANZ Journal of Surgery"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic vascular disorder which is characterised by the development of arteriovenous malformations and telangiectasias. A key clinical manifestation is recurrent epistaxis. This study examined the impact of a dedicated HHT clinic in a major Australian tertiary hospital on epistaxis symptoms and subjective quality of life.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A multidisciplinary HHT clinic was established in 2015. All patients satisfied either genetic diagnosis or fulfilled Curacao criteria. A protocol based clinical assessment was performed and a standardised treatment regimen was implemented. Patients completed quality of life and epistaxis severity score (ESS) questionnaires at each review.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Participants of the dedicated clinic included 21 females (58.3%) and 15 males (41.7%), with a mean age of 49.0\u2009\u00b1\u200924.0\u2009years. The <jats:italic>ACVRL1<\/jats:italic> variant was the most common (<jats:italic>n<\/jats:italic>\u00a0=\u200920, 55.6%). A statistically significant reduction in epistaxis severity was noted between the baseline and second review (<jats:italic>P<\/jats:italic>\u00a0=\u20090.02) and was maintained to the third visit (<jats:italic>P<\/jats:italic>\u00a0=\u20090.015). Patients older than 50\u2009years demonstrate a consistently higher ESS than those less than 50\u2009years old (<jats:italic>P<\/jats:italic>\u00a0=\u20090.03). This trend is noted throughout the follow up period with the dedicated clinic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The introduction of a multidisciplinary, dedicated HHT clinic to provide enhanced assessment, monitored treatment regimen and greater access to care, resulted in improvement in the management of epistaxis and quality of life in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ans.17322","type":"journal-article","created":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T12:56:02Z","timestamp":1635771362000},"page":"499-504","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Improvement in epistaxis management: the experience of a dedicated hereditary haemorrhagic telangiectasia clinic"],"prefix":"10.1111","volume":"92","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0545-6616","authenticated-orcid":false,"given":"Rebecca","family":"Anning","sequence":"first","affiliation":[{"name":"Department of Otolaryngology Head and Neck Surgery John Hunter Hospital  New Lambton New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johnson","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology Head and Neck Surgery John Hunter Hospital  New Lambton New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Ronan","sequence":"additional","affiliation":[{"name":"General Genetics Service Hunter Genetics  Waratah New South Wales Australia"},{"name":"School of Medicine and Public Health The University of Newcastle  Newcastle New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jillian","family":"de Malmanche","sequence":"additional","affiliation":[{"name":"Hunter Haematology Unit Calvary Mater Hospital  Waratah New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rebecca","family":"Asher","sequence":"additional","affiliation":[{"name":"NHMRC Clinical Trials Centre University of Sydney  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8958-736X","authenticated-orcid":false,"given":"Tsu\u2010Hui (Hubert)","family":"Low","sequence":"additional","affiliation":[{"name":"Department of Head and Neck Surgery Chris O'Brien Lifehouse  Sydney New South Wales Australia"},{"name":"Central Clinical School University of Sydney  Sydney New South Wales Australia"},{"name":"Department of Otolaryngology Head and Neck Surgery Royal Prince Alfred Hospital  Camperdown New South Wales Australia"},{"name":"Faculty of Medicine and Health Sciences Macquarie University  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,11]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2018.193003"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3182136d32"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2009.35"},{"issue":"6","key":"e_1_2_7_5_1","first-page":"203","article-title":"Hereditary haemorrhagic telangiectasia: Pathophysiology","volume":"24","author":"Shovlin C","year":"2010","journal-title":"diagnosis and treatment"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e31802d8373"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.4193\/Rhin14.274"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20818"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anl.2015.12.001"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40463-017-0235-x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.7326\/M20-1443"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/bs.podrm.2017.02.006"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/coa.12821"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-48502-9"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40463-016-0116-8"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2017.31.4427"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajr.2008.22.3145"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0366-y"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2015.00035"}],"container-title":["ANZ Journal of Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ans.17322","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ans.17322","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ans.17322","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T05:04:44Z","timestamp":1698123884000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ans.17322"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,11]]},"references-count":18,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,3]]}},"alternative-id":["10.1111\/ans.17322"],"URL":"https:\/\/doi.org\/10.1111\/ans.17322","archive":["Portico"],"relation":{},"ISSN":["1445-1433","1445-2197"],"issn-type":[{"type":"print","value":"1445-1433"},{"type":"electronic","value":"1445-2197"}],"subject":[],"published":{"date-parts":[[2021,11]]},"assertion":[{"value":"2021-04-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-10-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-11-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}