{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T17:20:42Z","timestamp":1759339242875},"reference-count":39,"publisher":"AACN Publishing","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,6,1]]},"abstract":"<jats:p>Hereditary hemorrhagic telangiectasia is a rare, autosomal dominant genetic disease that causes abnormal growth of blood vessels and, subsequently, life-threatening arteriovenous malformations in vital organs. Epistaxis may be one of the initial clues that a patient has more serious, generalized arteriovenous malformations. Recommended treatment involves careful evaluation to determine the severity and risk of spontaneous rupture of the malformations and the management of various signs and symptoms. The disease remains undiagnosed in many patients, and health care providers may miss the diagnosis until catastrophic events happen in multiple family members. Prompt recognition of hereditary hemorrhagic telangiectasia and early intervention can halt the dangerous course of the disease. Critical care nurses can assist with early diagnosis within families with this genetic disease, thus preventing early death and disability.<\/jats:p>","DOI":"10.4037\/ccn2016270","type":"journal-article","created":{"date-parts":[[2016,6,1]],"date-time":"2016-06-01T08:16:10Z","timestamp":1464768970000},"page":"36-48","source":"Crossref","is-referenced-by-count":1,"title":["Hereditary Hemorrhagic Telangiectasia: A Primer for Critical Care Nurses"],"prefix":"10.4037","volume":"36","author":[{"given":"Kathleen M.","family":"Sacco","sequence":"first","affiliation":[{"name":"Kathleen M. Sacco is the director, Intensive Care Unit and Cardiopulmonary Services, Simi Valley Hospital, Simi Valley, California."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"Thomas W.","family":"Barkley","sequence":"additional","affiliation":[{"name":"Thomas W. Barkley, Jr, is president of Barkley and Associates, West Hollywood, California."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2471","reference":[{"issue":"3","key":"2019112612151301900_b1-0360036","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1177\/0009922813512893","article-title":"The curious case of hypoxemia","volume":"53","author":"Pai","year":"2014","journal-title":"Clin Pediatr"},{"key":"2019112612151301900_b2-0360036","unstructured":"Bergen T . Osler-Weber-Rendu disease. Healthline. http:\/\/www.healthline.com\/health\/osler-weber-rendu-syndrome#Overview1. Reviewed February 11, 2016. Accessed March 8, 2016."},{"issue":"8","key":"2019112612151301900_b3-0360036","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1503\/cmaj.081739","article-title":"Hereditary hemorrhagic telangiectasia","volume":"180","author":"Grand\u2019Maison","year":"2009","journal-title":"CMAJ"},{"issue":"1049","key":"2019112612151301900_b4-0360036","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1136\/postgradmedj-2012-131513","article-title":"Osler-Weber-Rendu syndrome simulating pulmonary metastasis","volume":"89","author":"Pereira e Silva","year":"2013","journal-title":"Postgrad Med J"},{"issue":"4","key":"2019112612151301900_b5-0360036","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1002\/lary.23736","article-title":"Efficacy of a topical sesame\/rose geranium oil compound in patients with hereditary hemorrhagic telangiectasia associated epistaxis","volume":"123","author":"Reh","year":"2013","journal-title":"Laryngoscope"},{"issue":"2","key":"2019112612151301900_b6-0360036","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1136\/jmg.2009.069013","article-title":"International guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia","volume":"48","author":"Faughnan","year":"2011","journal-title":"J Med Genet"},{"issue":"4","key":"2019112612151301900_b7-0360036","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1016\/j.ajem.2012.11.015","article-title":"Transient ischemic attack in hereditary hemorrhagic telangiectasia","volume":"31","author":"Gialdini","year":"2013","journal-title":"Am J Emerg Med"},{"issue":"4","key":"2019112612151301900_b8-0360036","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.jbiosc.2012.10.010","article-title":"Keloid pathogenesis via Drosophila similar to mothers against decapentaplegic (SMAD) signaling in a primary epithelial-mesenchymal in vitro model treated with biomedical-grade chitosan porous skin regenerating template","volume":"115","author":"Lim","year":"2013","journal-title":"J Biosci Bioeng"},{"issue":"5","key":"2019112612151301900_b9-0360036","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1002\/lary.23893","article-title":"Lifestyle and dietary influences on nosebleed severity in hereditary hemorrhagic telangiectasia","volume":"123","author":"Silva","year":"2013","journal-title":"Laryngoscope"},{"issue":"18","key":"2019112612151301900_b10-0360036","doi-asserted-by":"crossref","first-page":"1886","DOI":"10.1212\/WNL.0000000000001538","article-title":"Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study","volume":"84","author":"Donaldson","year":"2015","journal-title":"Neurology"},{"key":"2019112612151301900_b11-0360036","unstructured":"HHT Foundation International. Learn more about hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome). http:\/\/curehht.org\/wp-content\/uploads\/2010\/04\/Learn-More-About-HHT.pdf. Accessed March 8, 2016."},{"key":"2019112612151301900_b12-0360036","unstructured":"UCLA Radiology. Hereditary hemorrhagic telangiectasia (HHT) treatment program: about HHT. http:\/\/radiology.ucla.edu\/site.cfm?id=275. Accessed March 8, 2016."},{"issue":"2","key":"2019112612151301900_b13-0360036","first-page":"89","article-title":"Anemia and mucocutaneous telangiectasias","volume":"89","author":"Grillo","year":"2014","journal-title":"Am Fam Physician"},{"key":"2019112612151301900_b14-0360036","first-page":"191","article-title":"Optimal management of hereditary hemorrhagic telangiectasia","volume":"5","author":"Garg","year":"2014","journal-title":"J Blood Med"},{"issue":"5","key":"2019112612151301900_b15-0360036","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/j.amjcard.2013.04.052","article-title":"Angina pectoris or myocardial infarctions, pulmonary arteriovenous malformations, hereditary hemorrhagic telangiectasia, and paradoxical emboli","volume":"112","author":"Clark","year":"2013","journal-title":"Am J Cardiol"},{"issue":"1","key":"2019112612151301900_b16-0360036","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1007\/s00330-011-2243-y","article-title":"Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients","volume":"22","author":"Barral","year":"2012","journal-title":"Eur Radiol"},{"key":"2019112612151301900_b17-0360036","article-title":"Advanced introductory principles in pharmacology","volume-title":"Lecture presented at California State University, Los Angeles","author":"Barkley","year":"2014"},{"issue":"4","key":"2019112612151301900_b18-0360036","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1007\/s11739-011-0531-9","article-title":"Liver involvement in hereditary hemorrhagic telangiectasia: can breath test unmask impaired hepatic first-pass effect?","volume":"7","author":"Candelli","year":"2012","journal-title":"Intern Emerg Med"},{"issue":"4","key":"2019112612151301900_b19-0360036","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1007\/s11739-012-0810-0","article-title":"The many faces of hereditary hemorrhagic telangiectasia: median arcuate ligament syndrome, arteriovenous malformation, and cerebral aneurysms","volume":"7","author":"Chick","year":"2012","journal-title":"Intern Emerg Med"},{"issue":"5","key":"2019112612151301900_b20-0360036","first-page":"1031","article-title":"Elevated factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism","volume":"98","author":"Shovlin","year":"2007","journal-title":"Thromb Haemost"},{"issue":"4","key":"2019112612151301900_b21-0360036","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1136\/thoraxjnl-2011-201076","article-title":"Low serum iron levels are associated with elevated plasma levels of coagulation factor VIII and pulmonary emboli\/deep venous thromboses in replicate cohorts of patients with hereditary haemorrhagic telangiectasia","volume":"67","author":"Livesey","year":"2012","journal-title":"Thorax"},{"issue":"4","key":"2019112612151301900_b22-0360036","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/joim.12098","article-title":"Immunological abnormalities associated with hereditary hemorrhagic telangiectasia","volume":"274","author":"Guilhem","year":"2013","journal-title":"J Intern Med"},{"key":"2019112612151301900_b23-0360036","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1186\/1750-1172-8-195","article-title":"Specific cancer rates may differ in patients with hereditary haemorrhagic telangiectasia compared to controls","volume":"8","author":"Hosman","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"12","key":"2019112612151301900_b24-0360036","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1111\/j.1540-8167.2012.02360.x","article-title":"Incomplete endothelialization of left atrial appendage occlusion device 10 months after implantation","volume":"23","author":"Massarenti","year":"2012","journal-title":"J Cardiovasc Electrophysiol"},{"issue":"3","key":"2019112612151301900_b25-0360036","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1002\/ajmg.a.35715","article-title":"Hereditary hemorrhagic telangiectasia: how accurate are the clinical criteria?","volume":"161A","author":"van Gent","year":"2013","journal-title":"Am J Med Genet A"},{"key":"2019112612151301900_b26-0360036","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1038\/ejhg.2009.35","article-title":"Hereditary hemorrhagic telangiectasia: a clinical and scientific review","volume":"17","author":"Govani","year":"2009","journal-title":"Eur J Hum Genet"},{"key":"2019112612151301900_b27-0360036","unstructured":"National Patient Travel Center. About the National Patient Travel Center. http:\/\/www.patienttravel.org\/. Accessed March 8, 2016."},{"issue":"2","key":"2019112612151301900_b28-0360036","doi-asserted-by":"crossref","first-page":"174","DOI":"10.3109\/00016489.2012.718097","article-title":"Argon plasma coagulation is an effective treatment for hereditary hemorrhagic telangiectasia patients with severe nosebleeds","volume":"133","author":"Pagella","year":"2013","journal-title":"Acta Otolaryngol"},{"key":"2019112612151301900_b29-0360036","unstructured":"Melville NA . Thalidomide for hemorrhagic telangiectasia epistaxis. http:\/\/www.medscape.com\/viewarticle\/826872. Published June 7, 2014. Accessed March 8, 2016."},{"issue":"8","key":"2019112612151301900_b30-0360036","doi-asserted-by":"crossref","first-page":"952.e1","DOI":"10.1016\/j.ajem.2014.02.005","article-title":"A novel approach to manage recurrent epistaxis in outpatients with hereditary hemorrhagic telangiectasia","volume":"32","author":"Cantone","year":"2014","journal-title":"Am J Emerg Med"},{"issue":"2","key":"2019112612151301900_b31-0360036","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1002\/lary.20757","article-title":"Hereditary hemorrhagic telangiectasia\/avastin","volume":"120","author":"Davidson","year":"2010","journal-title":"Laryngoscope"},{"issue":"6","key":"2019112612151301900_b32-0360036","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1002\/hed.23655","article-title":"Intranasal submucosal bevacizumab for epistaxis in hereditary hemorrhagic telangiectasia: a double-blind, randomized, placebo-controlled trial","volume":"37","author":"Riss","year":"2015","journal-title":"Head Neck"},{"issue":"3","key":"2019112612151301900_b33-0360036","doi-asserted-by":"crossref","first-page":"794","DOI":"10.4161\/mabs.28025","article-title":"ELLIPSE Study: a phase 1 study evaluating the tolerance of bevacizumab nasal spray in the treatment of epistaxis in hereditary hemorrhagic telangiectasia","volume":"6","author":"Dupuis-Girod","year":"2014","journal-title":"MAbs"},{"issue":"1","key":"2019112612151301900_b34-0360036","first-page":"85","article-title":"The effect of N-acetylcysteine on epistaxis and quality of life in patients with HHT: a pilot study","volume":"47","author":"de Gussem","year":"2009","journal-title":"Rhinology"},{"issue":"3","key":"2019112612151301900_b35-0360036","first-page":"14","article-title":"Hemorrhagic hereditary telangiectasia and arteriovenous malformations","volume":"11","author":"Chong","year":"2012","journal-title":"Int Stud J Nurse Anesth"},{"issue":"1","key":"2019112612151301900_b36-0360036","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1136\/thoraxjnl-2011-201027","article-title":"Flight-related complications are infrequent in patients with hereditary haemorrhagic telangiectasia\/pulmonary arteriovenous malformations, despite low oxygen saturations and anaemia","volume":"67","author":"Mason","year":"2012","journal-title":"Thorax"},{"issue":"9","key":"2019112612151301900_b37-0360036","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1111\/j.1471-0528.2008.01786.x","article-title":"Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services","volume":"115","author":"Shovlin","year":"2008","journal-title":"BJOG"},{"issue":"2","key":"2019112612151301900_b38-0360036","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.carj.2013.02.007","article-title":"Cumulative radiation dose in patients with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations","volume":"65","author":"Hanneman","year":"2014","journal-title":"Can Assoc Radiol J"},{"issue":"3","key":"2019112612151301900_b39-0360036","doi-asserted-by":"crossref","first-page":"206","DOI":"10.3109\/09638288.2012.690500","article-title":"Living with hereditary hemorrhagic telangiectasia: coping and psychological distress\u2014a cross-sectional study","volume":"35","author":"Geirdal","year":"2013","journal-title":"Disabil Rehabil"}],"container-title":["Critical Care Nurse"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/aacnjournals.org\/ccnonline\/article-pdf\/36\/3\/36\/116981\/36.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/aacnjournals.org\/ccnonline\/article-pdf\/36\/3\/36\/116981\/36.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,21]],"date-time":"2020-09-21T11:51:44Z","timestamp":1600689104000},"score":1,"resource":{"primary":{"URL":"https:\/\/aacnjournals.org\/ccnonline\/article\/36\/3\/36\/20664\/Hereditary-Hemorrhagic-Telangiectasia-A-Primer-for"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,6,1]]},"references-count":39,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2016,6,1]]}},"URL":"https:\/\/doi.org\/10.4037\/ccn2016270","relation":{},"ISSN":["0279-5442","1940-8250"],"issn-type":[{"value":"0279-5442","type":"print"},{"value":"1940-8250","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,6,1]]}}}