{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T19:39:23Z","timestamp":1788205163221,"version":"build-2803163510"},"reference-count":51,"publisher":"Georg Thieme Verlag KG","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Laryngorhinootologie"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die heredit\u00e4re h\u00e4morrhagische Teleangiektasie (HHT; Morbus Osler-Weber-Rendu; Morbus Osler) ist mit einer weltweiten Pr\u00e4valenz von 1:5000\u20138000 eine seltene Erkrankung der Kapillaren, bei der sich arterioven\u00f6se Shunts bilden. Vorwiegend finden sich diese in der Nasenschleimhaut, im Magen-Darm-Trakt, in der Lunge, in der Leber und im zentralen Nervensystem. Leitsymptom der Erkrankung ist die Epistaxis. Die Therapie besteht aus einem mehrstufigen Behandlungskonzept, das neben Nasenpflege und der Lasertherapie in \u00f6rtlicher Bet\u00e4ubung auch medikament\u00f6se Therapien sowie operative Eingriffe in Vollnarkose umfasst. Zus\u00e4tzlich erfolgt ein Screening zur Erfassung der Beteiligung innerer Organe in enger interdisziplin\u00e4rer Zusammenarbeit mit den entsprechenden Fachabteilungen. Eine Behandlung der L\u00e4sionen im Bereich der (Schleim-) Haut, des Magen-Darm-Trakts und der Leber erfolgt symptomorientiert, wohingegen Gef\u00e4\u00dfmalformationen der Lunge und des zentralen Nervensystems aufgrund potenzieller und zum Teil bedrohlicher Komplikationen gelegentlich auch ohne subjektive Beschwerden behandlungsbed\u00fcrftig sind.<\/jats:p>","DOI":"10.1055\/a-1220-7045","type":"journal-article","created":{"date-parts":[[2020,9,28]],"date-time":"2020-09-28T22:38:44Z","timestamp":1601332724000},"page":"682-693","source":"Crossref","is-referenced-by-count":5,"title":["Diagnostik und Behandlung der heredit\u00e4ren h\u00e4morrhagischen Teleangiektasie"],"prefix":"10.1055","volume":"99","author":[{"given":"Caroline Theresa","family":"Seebauer","sequence":"additional","affiliation":[{"name":"HNO-Klinik, Universit\u00e4tsklinik Regensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Kuehnel","sequence":"additional","affiliation":[{"name":"HNO-Klinik, Universit\u00e4tsklinik Regensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wibke","family":"Uller","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr R\u00f6ntgendiagnostik, Universit\u00e4tsklinik Regensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Bohr","sequence":"additional","affiliation":[{"name":"HNO-Klinik, Universit\u00e4tsklinik Regensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kornelia Elisabeth","family":"Andorfer","sequence":"additional","affiliation":[{"name":"HNO-Klinik, Universit\u00e4tsklinik Regensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,9,28]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1111\/j.1478-3231.2006.01340.x","article-title":"Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations","volume":"26","author":"E Buscarini","year":"2006","journal-title":"Liver Int"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1016\/j.otc.2017.09.017","article-title":"Hereditary Hemorrhagic Telangiectasia","volume":"51","author":"T K\u00fchnel","year":"2018","journal-title":"Otolaryngol Clin North Am"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.otc.2017.09.006","article-title":"Etiology and Genetics of Congenital Vascular Lesions","volume":"51","author":"A Queisser","year":"2018","journal-title":"Otolaryngol Clin North Am"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1002\/ajmg.a.33206","article-title":"Overlapping spectra of SMAD4 mutations in juvenile polyposis (JP) and JP-HHT syndrome","volume":"152A","author":"C Gallione","year":"2010","journal-title":"Am J Med Genet A"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"2155","DOI":"10.1002\/ajmg.a.31450","article-title":"A fourth locus for hereditary hemorrhagic telangiectasia maps to chromosome 7","volume":"140","author":"P Bayrak-Toydemir","year":"2006","journal-title":"Am J Med Genet A"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1016\/j.ajhg.2013.07.004","article-title":"BMP9 Mutations Cause a Vascular-Anomaly Syndrome with Phenotypic Overlap with Hereditary Hemorrhagic Telangiectasia","volume":"93","author":"W L Wooderchak-Donahue","year":"2013","journal-title":"Am J Hum Genet"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/fgene.2015.00001","article-title":"Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era","volume":"6","author":"J McDonald","year":"2015","journal-title":"Front Genet"},{"key":"ref8","article-title":"Mouse models of hereditary hemorrhagic telangiectasia: recent advances and future challenges","volume":"6","author":"S Tual-Chalot","year":"2015","journal-title":"Front Genet"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1007\/s00405-016-4431-5","article-title":"Influence of temporary nasal occlusion (tNO) on epistaxis frequency in patients with hereditary hemorrhagic telangiectasia (HHT)","volume":"274","author":"K EC Wirsching","year":"2017","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"135","DOI":"10.4193\/Rhin16.315","article-title":"Nasal closure for severe hereditary haemorrhagic telangiectasia in 100 patients. 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