{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,2]],"date-time":"2022-04-02T08:42:08Z","timestamp":1648888928627},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2018,4,23]],"date-time":"2018-04-23T00:00:00Z","timestamp":1524441600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["HNO"],"published-print":{"date-parts":[[2018,5]]},"DOI":"10.1007\/s00106-018-0503-8","type":"journal-article","created":{"date-parts":[[2018,4,23]],"date-time":"2018-04-23T06:56:03Z","timestamp":1524466563000},"page":"405-414","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Diagnostik und Therapie des\u00a0M.\u00a0Osler"],"prefix":"10.1007","volume":"66","author":[{"given":"F.","family":"Haubner","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"K\u00fchnel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2018,4,23]]},"reference":[{"key":"503_CR1","first-page":"769","volume":"1","author":"HG Sutton","year":"1864","unstructured":"Sutton HG (1864) Epistaxis as an indication of impaired nutrition, and of degeneration of the vascular system. Med Mirror 1:769\u2013781","journal-title":"Med Mirror"},{"issue":"2","key":"503_CR2","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1136\/jmg.2009.069013","volume":"48","author":"ME Faughnan","year":"2011","unstructured":"Faughnan ME et\u00a0al (2011) International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J\u00a0Med Genet 48(2):73\u201387","journal-title":"J Med Genet"},{"issue":"4","key":"503_CR3","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1111\/1523-1747.ep12555569","volume":"95","author":"IM Braverman","year":"1990","unstructured":"Braverman IM, Keh A, Jacobson BS (1990) Ultrastructure and three-dimensional organization of the telangiectases of hereditary hemorrhagic telangiectasia. J\u00a0Invest Dermatol 95(4):422\u2013427","journal-title":"J Invest Dermatol"},{"issue":"6","key":"503_CR4","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1016\/j.blre.2010.07.001","volume":"24","author":"CL Shovlin","year":"2010","unstructured":"Shovlin CL (2010) Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment. Blood Rev 24(6):203\u2013219","journal-title":"Blood Rev"},{"key":"503_CR5","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3389\/fgene.2015.00001","volume":"6","author":"J McDonald","year":"2015","unstructured":"McDonald J et\u00a0al (2015) Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a\u00a0new era. Front Genet 6:1","journal-title":"Front Genet"},{"issue":"6","key":"503_CR6","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1042\/CS20110086","volume":"121","author":"JF Santibanez","year":"2011","unstructured":"Santibanez JF, Quintanilla M, Bernabeu C (2011) TGF-beta\/TGF-beta receptor system and its role in physiological and pathological conditions. Clin Sci 121(6):233\u2013251","journal-title":"Clin Sci"},{"issue":"2","key":"503_CR7","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1136\/jmg.2005.030833","volume":"43","author":"SA Abdalla","year":"2006","unstructured":"Abdalla SA, Letarte M (2006) Hereditary haemorrhagic telangiectasia: current views on genetics and mechanisms of disease. J\u00a0Med Genet 43(2):97\u2013110","journal-title":"J Med Genet"},{"issue":"2","key":"503_CR8","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1002\/ajmg.a.33206","volume":"152A","author":"C Gallione","year":"2010","unstructured":"Gallione C et al (2010) Overlapping spectra of SMAD4 mutations in juvenile polyposis (JP) and JP-HHT syndrome. Am J Med Genet\u00a0A 152A(2):333\u2013339","journal-title":"Am J Med Genet A"},{"issue":"3","key":"503_CR9","doi-asserted-by":"publisher","first-page":"530","DOI":"10.1016\/j.ajhg.2013.07.004","volume":"93","author":"WL Wooderchak-Donahue","year":"2013","unstructured":"Wooderchak-Donahue WL et al (2013) BMP9 mutations cause a\u00a0vascular-anomaly syndrome with phenotypic overlap with hereditary hemorrhagic telangiectasia. Am J Hum Genet 93(3):530\u2013537","journal-title":"Am J Hum Genet"},{"issue":"21","key":"503_CR10","doi-asserted-by":"publisher","first-page":"7723","DOI":"10.1073\/pnas.1318761111","volume":"111","author":"K Kawasaki","year":"2014","unstructured":"Kawasaki K et\u00a0al (2014) Genetic variants of Adam17 differentially regulate TGFbeta signaling to modify vascular pathology in mice and humans. Proc Natl Acad Sci USA 111(21):7723\u20137728","journal-title":"Proc Natl Acad Sci USA"},{"key":"503_CR11","doi-asserted-by":"publisher","first-page":"25","DOI":"10.3389\/fgene.2015.00025","volume":"6","author":"S Tual-Chalot","year":"2015","unstructured":"Tual-Chalot S, Oh SP, Arthur HM (2015) Mouse models of hereditary hemorrhagic telangiectasia: recent advances and future challenges. Front Genet 6:25","journal-title":"Front Genet"},{"issue":"8","key":"503_CR12","doi-asserted-by":"publisher","first-page":"1425","DOI":"10.1161\/CIRCRESAHA.109.211037","volume":"106","author":"M Mahmoud","year":"2010","unstructured":"Mahmoud M et\u00a0al (2010) Pathogenesis of arteriovenous malformations in the absence of endoglin. Circ Res 106(8):1425\u20131433","journal-title":"Circ Res"},{"issue":"9","key":"503_CR13","doi-asserted-by":"publisher","first-page":"1174","DOI":"10.1288\/00005537-199409000-00023","volume":"104","author":"JL Gluckman","year":"1994","unstructured":"Gluckman JL, Portugal LG (1994) Modified Young\u2019s procedure for refractory epistaxis due to hereditary hemorrhagic telangiectasia. Laryngoscope 104(9):1174\u20131177","journal-title":"Laryngoscope"},{"issue":"1","key":"503_CR14","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1161\/CIRCRESAHA.112.275586","volume":"112","author":"EM Garrido-Martin","year":"2013","unstructured":"Garrido-Martin EM et al (2013) Vascular injury triggers Kruppel-like factor 6 mobilization and cooperation with specificity protein 1 to promote endothelial activation through upregulation of the activin receptor-like kinase 1 gene. Circ Res 112(1):113\u2013127","journal-title":"Circ Res"},{"issue":"12","key":"503_CR15","doi-asserted-by":"publisher","first-page":"4001","DOI":"10.1182\/blood.V100.12.4001","volume":"100","author":"LM Botella","year":"2002","unstructured":"Botella LM et al (2002) Transcriptional activation of endoglin and transforming growth factor-beta signaling components by cooperative interaction between Sp1 and KLF6: their potential role in the response to vascular injury. Blood 100(12):4001\u20134010","journal-title":"Blood"},{"issue":"1","key":"503_CR16","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1002\/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P","volume":"91","author":"CL Shovlin","year":"2000","unstructured":"Shovlin CL et al (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 91(1):66\u201367","journal-title":"Am J Med Genet"},{"issue":"3","key":"503_CR17","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1002\/ajmg.1320320302","volume":"32","author":"H Plauchu","year":"1989","unstructured":"Plauchu H et al (1989) Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 32(3):291\u2013297","journal-title":"Am J Med Genet"},{"issue":"7","key":"503_CR18","doi-asserted-by":"publisher","first-page":"860","DOI":"10.1038\/ejhg.2009.35","volume":"17","author":"FS Govani","year":"2009","unstructured":"Govani FS, Shovlin CL (2009) Hereditary haemorrhagic telangiectasia: a\u00a0clinical and scientific review. Eur J Hum Genet 17(7):860\u2013871","journal-title":"Eur J Hum Genet"},{"issue":"4","key":"503_CR19","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1159\/000479632","volume":"94","author":"S Dupuis-Girod","year":"2017","unstructured":"Dupuis-Girod S, Cottin V, Shovlin CL (2017) The lung in hereditary hemorrhagic telangiectasia. Respiration 94(4):315\u2013330","journal-title":"Respiration"},{"issue":"4","key":"503_CR20","doi-asserted-by":"publisher","first-page":"1891","DOI":"10.1007\/s00405-016-4431-5","volume":"274","author":"KEC Wirsching","year":"2017","unstructured":"Wirsching KEC, Haubner F, Kuhnel TS (2017) Influence of temporary nasal occlusion (tNO) on epistaxis frequency in patients with hereditary hemorrhagic telangiectasia (HHT). Eur Arch Otorhinolaryngol 274(4):1891\u20131896","journal-title":"Eur Arch Otorhinolaryngol"},{"issue":"9","key":"503_CR21","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1007\/s001060050141","volume":"45","author":"JA Werner","year":"1997","unstructured":"Werner JA et al (1997) Treatment of recurrent epistaxis in Rendu-Osler-Weber disease. HNO 45(9):673\u2013681","journal-title":"HNO"},{"issue":"8","key":"503_CR22","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1055\/s-2007-997467","volume":"76","author":"JA Werner","year":"1997","unstructured":"Werner JA et\u00a0al (1997) Nd:YAG laser therapy of recurrent epistaxis in hereditary hemorrhagic telangiectasia. Laryngorhinootologie 76(8):495\u2013501","journal-title":"Laryngorhinootologie"},{"issue":"2","key":"503_CR23","doi-asserted-by":"publisher","first-page":"153","DOI":"10.21053\/ceo.2016.00318","volume":"10","author":"KEC Wirsching","year":"2017","unstructured":"Wirsching KEC, Kuhnel TS (2017) Update on clinical strategies in hereditary hemorrhagic telangiectasia from an ENT point of view. Clin Exp Otorhinolaryngol 10(2):153\u2013157","journal-title":"Clin Exp Otorhinolaryngol"},{"issue":"2","key":"503_CR24","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1160\/TH06-07-0373","volume":"97","author":"LA Fernandez","year":"2007","unstructured":"Fernandez LA et al (2007) Therapeutic action of tranexamic acid in hereditary haemorrhagic telangiectasia (HHT): regulation of ALK-1\/endoglin pathway in endothelial cells. Thromb Haemost 97(2):254\u2013262","journal-title":"Thromb Haemost"},{"issue":"3","key":"503_CR25","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1160\/TH09-07-0425","volume":"103","author":"V Albinana","year":"2010","unstructured":"Albinana V et\u00a0al (2010) Estrogen therapy for hereditary haemorrhagic telangiectasia (HHT): effects of raloxifene, on endoglin and ALK1 expression in endothelial cells. Thromb Haemost 103(3):525\u2013534","journal-title":"Thromb Haemost"},{"issue":"5","key":"503_CR26","doi-asserted-by":"publisher","first-page":"833","DOI":"10.1124\/mol.110.067447","volume":"79","author":"V Albinana","year":"2011","unstructured":"Albinana V et al (2011) Immunosuppressor FK506 increases endoglin and activin receptor-like kinase 1 expression and modulates transforming growth factor-beta1 signaling in endothelial cells. Mol Pharmacol 79(5):833\u2013843","journal-title":"Mol Pharmacol"},{"issue":"3","key":"503_CR27","doi-asserted-by":"publisher","first-page":"226","DOI":"10.7326\/0003-4819-144-3-200602070-00030","volume":"144","author":"AI Skaro","year":"2006","unstructured":"Skaro AI, Marotta PJ, McAlister VC (2006) Regression of cutaneous and gastrointestinal telangiectasia with sirolimus and aspirin in a\u00a0patient with hereditary hemorrhagic telangiectasia. Ann Intern Med 144(3):226\u2013227","journal-title":"Ann Intern Med"},{"issue":"10","key":"503_CR28","doi-asserted-by":"publisher","first-page":"933","DOI":"10.1080\/14728222.2017.1365839","volume":"21","author":"L Ruiz-Llorente","year":"2017","unstructured":"Ruiz-Llorente L et al (2017) Endoglin and alk1 as therapeutic targets for hereditary hemorrhagic telangiectasia. Expert Opin Ther Targets 21(10):933\u2013947","journal-title":"Expert Opin Ther Targets"},{"issue":"4","key":"503_CR29","doi-asserted-by":"publisher","first-page":"H959","DOI":"10.1152\/ajpheart.01251.2009","volume":"299","author":"JM Lopez-Novoa","year":"2010","unstructured":"Lopez-Novoa JM, Bernabeu C (2010) The physiological role of endoglin in the cardiovascular system. Am J Physiol Heart Circ Physiol 299(4):H959\u201374","journal-title":"Am J Physiol Heart Circ Physiol"}],"container-title":["HNO"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00106-018-0503-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00106-018-0503-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00106-018-0503-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,22]],"date-time":"2019-04-22T23:37:15Z","timestamp":1555976235000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00106-018-0503-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,4,23]]},"references-count":29,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,5]]}},"alternative-id":["503"],"URL":"https:\/\/doi.org\/10.1007\/s00106-018-0503-8","relation":{},"ISSN":["0017-6192","1433-0458"],"issn-type":[{"value":"0017-6192","type":"print"},{"value":"1433-0458","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,4,23]]},"assertion":[{"value":"23 April 2018","order":1,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"F.\u00a0Haubner und T.\u00a0K\u00fchnel geben an, dass kein Interessenkonflikt besteht.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}},{"value":"F\u00fcr den hier vorliegenden Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgef\u00fchrt. F\u00fcr die im Beitrag aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics"}}]}}